March 1995

Lichen Sclerosus

Jeffrey J. Meffert, Brian M. Davis, and Ronald E. Grimwood.

Lichen sclerosus, usually appearing in the dermatologic literature under the names of lichen sclerosus et atrophicus, balanitis xerotica obliterans, and kraurosis vulvae, is an inflammatory disease with a multifactorial origin. A past association of lichen sclerosus and genital squamous cell carcinoma is not as close as once thought. Once considered primarily a surgical problem, especially when the genitals are involved, lichen sclerosus will respond to a variety of systemic and topical therapies.

[CIRP Note: The figures supplied with the article are not included in this file.]

Lichen sclerosus (LS), usually reported in the dermatologic literature as lichen sclerosus et atrophicus, is an inflammatory disease of unknown cause and incompletely characterized pathogenesis. Beginning with the initial case reports at the turn of the century, there has been much confusion in the literature because of the plethora of terms used to describe LS. Different names have been applied depending on the lesional location and the specialty of the author. More than 15 years ago, gynecologists standardized the terminology used in reporting vulvar dystrophies, including LS. Because the majority of reported cases of LS involve the female genitalia and because of the multiplicity of synonyms for LS one of the factors that makes the body of LS-related literature less accessible, we recommend that the gynecologists' term lichen sclerosus be used in future reports of LS, lichen sclerosus et atrophicus, and balanitis xerotica obliterans (BXO). Interest in LS remains high because of the pervasive, erroneous concept that LS itself is a premalignant condition.

    Hallopeau is usually credited with the first clinical description of what was to be later called lichen sclerosus et atrophicus. In 1887 he described a patient with coalescent papules on the trunk and forearms who also had pruritis and lichenification of the vulva.1However, in 1875 Weir described a patient with vulvar and oral "ichthyosis" and provided an excellent clinical description of LS, possibly the first published case of this disease.2During the next 11 years Hallopeau described three more cases of what he believed to be a unique form of atrophic lichen planus.3-5Darier6commented on Hallopeau's original case, but it was not until 1892 that Darier7formally described the typical histological features of LS. In the decades after Hallopeau's description many noteworthy independent observers "discovered" LS and applied their own names to what, in retrospect, was clinically and histologically the same disease. Some of the more frequent and widely published early synonyms for LS include lichen plan atrophique (Hallopeau,11887), lichen plan scléreux (Darier,71892), Kartenblattförmige Sklerodermie ("playing card" or "cardboard-like" scleroderma; Unna,81894), Weissflecken Dermatose ("white spot disease"; Westberg,91901), lichen albus (von Zumbusch, 10, 1901), lichen planus sclerosus et atrophicus (Montgomery and Ormsby,111907), dermatitis lichenoides chronica atrophicans (Csillag,121909), kraurosis vulvae (Breisky,131885), and balanitis xerotica obliterans (Stühmer,141928).
    Much of the world literature on LS is confounded with other entities under such terms as circumscribed scleroderma, leukoplakic vulvitis, primary atrophic vulvitis, atrophic and bullous lichen planus, acrodermatitis chronica atrophicans, and bullous scleroderma. Although most of the dermatologic literature reports "lichen sclerosus et atrophicus," the shortened "lichen sclerosus" has appeared sporadically since the 1920s. However, Friedrich15in 1976 argued that LS was more "dystrophic" than "atrophic." "Lichen sclerosus" was formally adopted by the International Society for the Study of Vulvar Disease16and "et atrophicus" was abandoned in the gynecologic literature. In accordance with this standardization in nomenclature, the term lichen sclerosus will be used unless it is unclear that all data and opinions in some older reports refer exclusively to LS and not a blend of LS and other disease entities (often the case in discussions of "leukoplakia" and "kraurosis").
    The controversy about the relation of LS to lichen planus (LP) and scleroderma began early and continues to this day. Hallopeau believed that LS is a form of LP because of occasional oral findings.17Others counter with the histologic features of oral lesions so typical of LS that the existence of "oral LS" is recognized18-25even as the sole manifestation.26Until recently, some authors still argued for a close relation of LS with LP, proposing that if enough sections were cut in a biopsy specimen, typical LP would be found.27Others, while accepting LS as a distinct entity, suggest that Hallopeau's original cases were really atrophic LP.17Although most recent reports do not link LS and LP except as coincidental findings, the association of LS and morphea (localized scleroderma) remains a topic of research and debate and will be discussed in detail later.
    Divisive to the establishment of consistent principles of diagnosis and treatment was the parallel work on the nature and therapy of different medical disciplines. The gynecologic literature predates Hallopeau with Breisky's description of "kraurosis vulvae" in 1885.13The urologic literature dates back to Stühmer's description14of balanitis xerotica obliterans in 1928. Ormsby and Mitchell28first connected LS with kraurosis vulvae in 1920, and Freeman and Laymon,29,30made the same association with balanitis xerotica obliterans in 1941. Table I chronicles key events leading to our current understanding of LS.

Table 1: Significant events in the history of LS
Year Event Key reference no.(s)
1875 Weir's report of possible vulvar and/or oral LS as "ichthyosis"
1885 Breisky describes kraurosis vulvae 13
1887 Hallopeau describes series of extragenital LS (and one possible vulvar case) 1, 3-5
1892 Darier formally describes classic histopathology of LS 6, 7
1900-present  Concept that scleroderma and LS are closely related 72, 73, 222, 227, 231, 290, 358
1901 Pediatric LS described 9, 53, 95, 96, 101, 103
1913-present  Concept that scleroderma is not closely related to LS 45, 145, 148, 158, 238, 240, 281
1920 Taussig establishes vulvectomy as treatment of choice for kraurosis vulvae, a premalignant condition 93, 183-185; also 79, 186, 191, 196
1927 Kyrle defines LS ("white spot disease") as entity sui generis 359
1928 Stühmer describes balanitis xerotica obliterans as postcircumcision phenomenon 14
1936 Retinoids (vitamin A) used in LS 47, 195, 341, 349, 352, 353
1945 Testosterone used in genital LS 87, 92, 160, 254, 337, 338, 342
1961 Modern use of corticosteroids 21, 102, 165, 254, 337, 338, 342
1966 Jeffcoate presents argument against vulvectomy for simple LS 119; also 51, 198, 209
1971 Progesterone used in LS 254, 338, 344, 345
1971 Wallace defines clinical factors and epidemiology of LS for all later reports 21
1976 Friedrich defines LS as a dystrophic, not atrophic condition; "et atrophicus" dropped 15
1976 International Society for Study of Vulvar Disease classification system. "Kraurosis" and "leukoplakia" no longer to be used 16, 181, 182
1980 Fluourinated and superpotent steroids used in LS 54, 56, 96, 101-103, 254
1981 Studies into HLA serotypes and LS 44, 217, 273, 275-77, 279
1984 Etretinate and acetretin used in LS 25, 149, 343, 354-357
1987 LS linked with Borrelia infection 232, 233, 256, 289, 290

    LS can affect all age groups with reported onset from 6 months of age to late adulthood.21,31,32Most patients described in the literature are Caucasian, but the fact that most studies come out of Great Britain and the United States may create a reporting bias. LS has been reported in native Africans,33,34Oriental patients,35and other dark-skinned patients.36-41The majority are women, but that reflects the intensive study of vulvar "leukoplakia" over the years. The reported female-to-male ranges from nearly 10:1,42,43to nearer 5:1,21, 44-47to about 1:1.48,49More cases of purely genital involvement are reported than purely non-genital. However, firm epidemiological data are lacking for many reasons. Gynecologists and urologists do not generally perform a complete cutaneous examination, and dermatologists often exclude a genital examination. Furthermore, an anatomic definition of what constitutes "vulvar" versus "perineal" versus "genital" versus "groin" is lacking.50Table II summarizes cases known or suspected to be LS.

Table 2: A review of LS cases1-377(N = 5207)
Female 4280 
Male 691
Unspecified 236 Demographics in metabolic studies often not specified.
Genital 4308 Includes labial, perineal, perianal, and any penile location.
Extragential 805 Neck, shoulders, upper trunk most common sites reported.
Both 355 Excludes cases in which site is uncertain.
Associated Factors
Cancer 283 Almost all are SCC of the vulva. Includes unknown number of other vulvar dystrophies.
Autoimmune disease 291 Not always reported or sought. Thyroid disease, vitiligo, diabetes, and pernicious anemia most common.
Autoantibodies 316 Not always reported or sought. Thyroid antimicrosomal, gastric parietal cell, and antinuclear autobodies most common.

Data expressed as total number of LS cases within each category. SCC, Squamous cell carcinoma.

    In women, prepubertal, perimenopausal, and postmenopausal vulvar and perineal involvement are most common. Symptoms include pruritis, burning pain, dyspareunia, dysuria, vaginal discharge, anal or genital bleeding,41, 51,52 labial stenosis or fusion,53,54 and, especially in children, constipation.55,56 Most cases reported in men occur on the glans penis and/or prepuce in prepubertal boys and middle-aged men. Male symptoms include sudden phimosis in a previously retractable foreskin,57 adhesions to the glans,29 decreased sensation on the glans, painful erections, meatal stenosis, dysuria, urethral discharge, and urinary obstruction.58-61 Ulceration of the glans can occur, even after circumcision.21,52
    Extragenital LS is most common on the neck and shoulders and is generally asymptomatic in both sexes. Pruritis occasionally occurs. Less common presentations include infraorbital LS,62 scarring alopecia,63 involvement of the palms and/or soles,64-68 appearance in acrochordons,69 or on the scrotum.70 The latter is surprisingly rare considering the frequency of genital labial involvement.71,72 Borda et al.73 proposes a "vitiligoid" variant in which pigmentary changes exist without papules and plaques. Oral LS is usually asymptomatic,18 although not invariably.22
    Typically, the eruption of LS begins as white, polygonal papules that coalesce into plaques. The appearance of being "splashed with whitewash" (Fig. 1) led to the early appellation of "white spot scleroderma."74 Characteristic are comedo-like plugs or evenly spaced dells (Fig. 2) in the surface of the plaque that correspond to appendageal ostia. The grouping of dells in coalescent papules has been described as cribriform or corymbiform.75 The plugs and dells may disappear with time as the lesion ages, leaving a smooth, often porcelain-white plaque. Milia may occasionally be apparent.76 Early LS may have a vesicular component,77 and early genital LS may be characterized by edema of the clitoral foreskin78 as well as labial telangiectases and purpura.21 Fissuring may occur in the perineal midline and other intertriginous areas.79 Bullous LS, often accompanied by intralesional hemorrhage,73,80-82 may be localized or generalized.83-86 The frenulum may be obliterated,87,88 and the glans may have a mottled appearance (Fig. 3). A sclerotic white ring at the preputial opening is diagnostic.57,88-90 Late LS has been described as "verrucous" on the elbows91 and other sites24, 73 and "hyperkeratotic" on the knees.40 However, such findings in a genital location would suggest other dystrophic or infectious diseases. Late LS in women is characterized by obliteration of the labia minora and periclitoral structures.21,92 Many terms are used to describe the involvement of labial, perineal, and anal areas, including "figure-8,"55 "keyhole,"55 "hourglass,"93,94 and the more poetic "butterfly" and "lotus flower"92 (Fig. 4).

Figure 1: Extragenital lichen sclerosus, gross morphology.
 (to be added when I can get the journal out of the library)

Figure 2: Extragenital lichen sclerosus.
 (to be added when I can get the journal out of the library)

Figure 3: Male genital lichen sclerosus. Irregular mottling of glans.
 (to be added when I can get the journal out of the library)

Figure 4: Female genital lichen sclerosus (early). "Figure-8" dystrophy with fusion of clitoral hood, and obliteration of labia minora.
 (to be added when I can get the journal out of the library)

LS in children is histologically identical to that in adults.55 However, it represents a special subset of LS for several reasons. Ten percent to 15% of LS cases occur in children, the majority of which involve the female genitalia.52 Again, the incidence is uncertain because of delay or failure to diagnose LS, masking of LS by secondary changes,95 a tendency for spontaneous involution, and a reluctance to seek medical care for what may be perceived as child sexual abuse.96 Because of the traumatic appearance of hemorrhagic and bullous LS, it is discussed in the pediatric literature as an entity often confused with sexual abuse.21, 97-103 However, it cannot be assumed that the diagnosis of LS excludes sexual abuse. In a 7-year-old girl initial improvement with treatment was followed by dramatic worsening and subsequent confirmation of sexual abuse.104
    At least half of the cases of genital LS in girls will involute by menarche,21, 105,106 and the earlier the onset of LS, the more likely the resolution.43 However, not all will resolve,101,107 and despite "resolution," scarring and pigmentary changes may persist.21,52,56,105,108 Many women with LS will recall childhood symptoms of pruritis. However, it is unclear whether LS in these women represents persistence, recurrence, or is unrelated to this history.21,103 Extragenital involvement may43 or may not96 be predictive of resolution. Spontaneous resolution, when it occurs, is attributed to a changing hormonal milieu and increasing endogenous androgen levels. However, because most patients also receive at least symptomatic therapy, few resolutions are truly "spontaneous."

    LS exhibits the isomorphic or "Koebner" phenomenon. Stühmer14 and Madden109 considered circumcision the cause of LS of the glans penis. However, most believe that trauma or chronic infection in the uncircumcised state leads to LS.58,110,111 LS has appeared on the penis after surgery for squamous cell carcinoma112 and after correction of genital abnormalities in a case of gonadal dysgenesis.113 Bale et al.114 described seven boys in whom LS developed after surgery to correct hypospadias.
    The isomorphic phenomenon may also contribute to vulvar LS.115 Inciting factors include "warmth and moisture,"116low-grade infections including pinworms,95,106,117,118 "tight" or nylon underwear,119 trauma from bicycle riding,55 superficial radiation therapy for pruritis,120 or repeated masturbation.121 Postoperative "Koebnerization" is often cited as a reason for the high recurrence rate after vulvectomy to treat LS.51,122,123
    LS may also appear in extragenital surgical sites,53,80,124 such as cholecystectomy69,125 and other surgical scars.126-128 In two patients LS of the trunk occurred after radiation therapy for breast cancer as much as 8 years later.129 Solar radiation and sunburn were reported to induce LS in at least 3 patients.34,43,130 LS appeared at the site of old scald burns decades later in another patient.131 One case involved elbow plaques of LS believed to be caused by trauma and rubbing.132
    The concept that friction from clothing may cause LS has been advanced several times, for example, brassiere straps suspected of causing subclinical shoulder trauma eventuating in LS.133,134 In fact, most LS cases reported in the early dermatologic literature, including Hallopeau's, occurred on the back and shoulders.135,136 The contribution of solar radiation in these cases in unknown. Friction as the stimulus of the isomorphic phenomenon is not confined to irritation by external agents. Many consider the trauma of skin rubbing against skin the ultimate trigger of genital and extragenital LS.69,125,137

    The characteristic histologic features of LS is critical in identifying cases reported under other names. Although variations exist because of lesion duration, the overall appearance is surprisingly consistent despite differences in patient age and lesion location.138-141 A compact orthohyperkeratosis usually underlies a markedly thinned epidermis. The hyperkeratosis usually overlies a markedly thinned epidermis. The hyperkeratosis may be such that the stratum corneum is thicker than the remainder of the epidermis (Fig. 5). Vacuolar degeneration of the basal layer is usual, especially in earlier lesions. When extensive, it may cause bulla formation (Fig. 6). Acanthosis, unless it is a part of a superimposed lichen simplex chronicus,139,140 is not a feature of LS, nor is cellular atypia.142,143 These findings in genital lesions suggest a hyperplastic or mixed dystrophy that may have malignant potential. Follicular occlusion by a cornified plug earlier in the course may eventuate in atrophy and disappearance of appendageal surfaces, including apocrine glands144 (Fig. 7). Most striking are the morphologic changes in the papillary dermis that becomes homogenized and edematous early and hyalinized and sclerotic later (Fig. 8). The superficial vascular plexus is pushed deeper so that it appears to be in the mid dermis.140

Figure 5: Lichen sclerosus. Compact hyperkeratosis thicker than effaced epidermis. Clefting and hemorrhage within homogenized papillary dermis. (Original maginification, X125).
 (to be added when I can get the journal out of the library)

Figure 6: Lichen sclerosus. Lichenoid infiltrate and dilated vessels underlying a hemorrhagic subepidermal bulla. (Original magnification, X125.)
 (to be added when I can get the journal out of the library)

Figure 7: 
 (to be added when I can get the journal out of the library)

Figure 8: 
 (to be added when I can get the journal out of the library)

    Investigation into the nature of the papillary dermal changes may provide clues to the cause of LS. Wallace21 did not believe special stains were particularly helpful. However, others have used special stains and techniques to define the pathologic changes more accurately. Increased ground substance145,146 composed of neutral147 and acid139 mucopolysaccharides is evident in the edematous papillary dermis. Most agree the elastic tissue of the papillary dermis degenerates to the point of complete absence in some cases.22, 53,105,148-152 However, Mann and Cowan153 found normal to increased amounts of elastin in an ultrastructural examination of extragenital LS. They conjectured that elastin was modified so as to stain poorly with orcein. Other ultrastructural studies have shown both degeneration of subepidermal elastin and changes in collagen.95,138,146,148,154,155 Type IV collagen and basement membrane itself become fragmented;95,114,145,146,154 focal thickening of reduplicated basement membrane can also be seen.147,153 In the papillary dermis collagen types I and III show variation in fiber diameter and gross alterations in alignment progressing to disintegration and eventual loss.139,149 Evidence of collagen regeneration is seen in treated LS.149 Microscopic evidence is of an active collagen degeneration and regeneration cycle in static and untreated LS belies the old appellation "atrophicus."95,138,146,153-155  This cycle of destruction and regeneration causes sclerosis in late lesions. Sánchez at al.151 described the histologic curiosity of vulvar LS with loss of papillary dermis elastin coexisting with an underlying nevus elasticus, an elastic tissue hamartoma characterized by dense, mid-dermal elastic fibers.
    A lichenoid mononuclear infiltrate may be dense and extensive or relatively sparse and patchy, especially in older lesions. Plasma cells have been reported;88,114 others believe this is more likely in similar diseases such as acrodermatitis chronica atrophicans.157 Eosinophils are an unusual finding,35,158 but, Janovski and Ames159 found significant tissue eosinophilia in 10% of their specimens. Mast cells may be increased,74, 95 decreased,160 or unchanged.154 Additional findings in LS include a characteristic nerve degeneration and regeneration in the papillary dermis141,161 and milia, thought to be of eccrine origin,114,162 in 15% of routine histopathologic specimens. Hewitt138 describes a detailed histologic grading system for vulvar LS that could be applied in other studies of LS.

    The treatment of genital LS has been primarily surgical until recently. Issues concerning male genital LS (BXO) are relatively straightforward. Some initially suggested restricting the diagnosis BXO to those cases in which there was meatal stenosis (seen in up to 25% of patients).87, 163 Meatal stenosis can occur without involvement of the remainder of the glans penis.88, 164 The term kraurosis penis appears sporadically but never achieved widespread use.109  Reports of LS in circumcised men52,57,165are conspicuous in a literature dominated by the concept that LS arises only in the incompletely or uncircumcised man59,110 caused by irritation, chronic balanitis, or subclinical trauma.
    An understanding of the relation between LS and the foreskin is confounded by the frequent failure to report indications and age at circumcision of patients with LS. When LS appears in circumcised men, a history of previous phimosis or adhesions is common. This many imply a preexisting LS condition because new onset or worsening phimosis and adhesion of the prepuce to the glans corona are key signs of male LS. LS, once considered rare in males,166 may be much more frequent than thought.61,114,167 Even in prepubertal boys, examination of foreskins submitted to histopathologic examination for various conditions (usually phimosis) reveals a significant percentage of LS (3.5% to 19%),57,60,61,114,168 often unsuspected. Skewing the statistics further, many normal, nonretractile prepubertal foreskins are submitted under the diagnosis of "phimosis," a condition that is diagnosed far more often that it truly occurs.169,170 If only truly sclerosed and stenotic foreskins were considered under this diagnosis, the incidence of LS can be as high as 95%.171-173
   Despite an oft-quoted association of penile LS and cancer,59 we found only nine clearly defined cases.112,174-176 At least two associated malignancies, Bowen's disease48,177 and verrucous carcinoma,178 may have contributing factors such as human papillomavirus (HPV) infection. Penile malignancies are associated with phimosis-related hygiene problems, and it may be the phimosis itself rather than the LS that caused it that is the true risk factor. Nevertheless, for penile LS circumcision is considered the treatment of choice for both therapeutic and prophylactic purposes. Medical therapies are generally reserved for those who have already been circumcised or those with persistent symptoms after surgery. After circumcision, it is not uncommon, especially in young adults, to have persistent plaques, discoloration, and occasional ulceration.21,89
    As in male genital LS, the prevalence of female genital LS is likely to be much higher than Wallace's oft-quoted 1 in 300 to 1 in 1000.21 Up to 12% of patients with idiopathic vulvadynia [sic] may have LS.179 Thorough autopsy studies and more liberal use of biopsy in patients with genital complaints could better define the prevalence of LS.180
    Adding to the confusion, the gynecologic literature on LS is replete with biases and taxonomic disorder. Reports contain various definitions of "lichen sclerosus," "kraurosis vulvae," "gross atrophy," "simple atrophy," "senile atrophy," and "vaginal dystrophy." The term leukoplakia is particularly obscure. Leukoplakia has been referred to as a clinical observation that became a microscopic diagnosis and, ultimately, a prognostic imperative.102 Most cases of kraurosis vulvae, and "simple," "gross," and "senile" atrophy, as well as many conditions termed leukoplakia represent LS.
    In 1976, the International Society for the Study of Vulvar Disease (SSDIV) [sic] classified LS as a vulvar dystrophy and devised a new classification system to address the ambiguities of nomenclature. When hyperplastic dystrophy, a term developed for this system, caused confusion on its own, a 1987 consensus modified the nomenclature once again (Table III). LS under both these systems is clinically and histologically identical to an identity long known to dermatologists as lichen sclerosus et atrophicus. The renaming and reclassification of LS was adopted by the gynecologic community, although it has been slow to find its way into the dermatologic and urologic literature.16,181,182 On the adoption of the ISSVD's recommendations, several terms were deleted from use. These included lichen sclerosus et atrophicus, leukoplakia, neurodermatitis, leukokeratosis, leukoplakic vulvitis, hyperplastic vulvitis, and kraurosis vulvae. The terms Bowen's disease, erythroplasia of Queyrat, and carcinoma simplex were deleted in favor of the inclusive term squamous cell carcinoma in situ. Paget's disease of the vulva remains in a separate disease.

Table 3: Nomenclature of vulvar disease
1976 terminology* 1987 terminology† Histologic description
LS LS Hyperkeratosis, effacement of epidermis, variable interface vacuolization, homogenization and edema of papillary dermis, and lichenoid infiltrate
Hyperplastic dystrophy without atypia Squamous cell hyperplasia Hyperkeratosis, acanthosis, epithelial hyperplasia without atypia and not attributable to a more specific dermatosis
Mixed dystrophy without atypia (Report both conditions above) Features of LS and hyperplastic dystrophy without atypia
(no equivalent) Other dermatoses Typical findings of a specific infectious or inflammatory disease (i.e., psoriasis, condyloma)
Hyperplastic dystrophy with atypia VIN Degree of atypia or grade of VIN graded according to degree of squamous proliferation, nuclear atypia and pleomorphism, individual cell keratinization, mitotic activity above the basal layer, and disordered maturation
Mixed dystrophy with atypia (Report both LS and VIN) Features of both LS and VIN

VIN, Vulvar intraepithelial neoplasia.
* According to the "Vulvar dystrophy" classification of the 1976 ISSVD committee.16,182
According to the "Nonneoplastic epithelial disorders of skin and mucosa" and "Vulvar intraepithelial neoplasia" classification of the 1987 ISSVD committee.181

    The impetus behind standardization of terminology was an effort to determine the true malignant potential of vulvar LS. Wallace's malignancy rate was 4%, which he believed was artificially reduced by the number of his patients who had already undergone vulvectomy.21 The dominant figure in linking LS with cancer was Taussig. He wrote a series of articles between 1920 and 1940 in which vulvas, removed for malignancy, were examined and found also to have evidence of "leukoplakia" in 73% of specimens.93, 183-185 Others proposed that LS represented an "advanced premalignant"186 condition with a "transformation" rate of 1% to 50% over the lifetime of the lesion.87, 147,180,187-193 On the basis of these data, the treatment of choice for LS and other "white lesions" of the vulva was vulvectomy. After the observation that LS had a recurrence rate of up to 78% after surgery,51,159 the recommendation was to try again,194 take wider surgical margins,195 or use frozen sections to ensure that all affected tissue was removed.187 In the literature of genital LS from 1920 through the mid 1970s, up to 30% of the patients enrolled in a study may have undergone vulvectomy for LS, leukoplakia, or even intractable pruritis. As late as 1987, descriptions of a technique for "prophylactic" total vulvectomy was described in the dermatologic literature.196 Vulvectomy is far from a benign procedure. Taussig185 reported an operative mortality rate of almost 10% that he attributed to the older age of his patients.
    Investigators subsequently noticed that cancers were most likely to arise in areas of hyperplastic or mixed dystrophy rather than contiguously with areas of clear-cut LS.51,177 In addition, an unknown number of patients with squamous cell carcinoma and LS in early reports received ionizing radiation as therapy. The contribution of radiation to future malignancy in the setting of LS is unknown.197 The prevailing thought is that although LS may be associated with other dystrophic changes with malignant potential, LS is not, itself, a premalignant condition and the association is more "casual than causal."41,47,140,198-199 Furthermore, it was noted that conservative therapy could reverse the histologic changes of LS200,201 and hyperplastic dystrophy. It was suggested that a therapeutic trial of conservative therapy with biopsies of persistent erosions, erythoplakia, or unusual-looking areas was more reasonable than immediate "prophylactic" vulvectomy.120, 202 Jeffcoate,119 in 1966, is usually credited with establishing the nonsurgical approach as appropriate for simple LS. However, sporadic reports of the "widely appreciated malignant potential of LS" appear to this day.
    Recent studies support the concept that LS is not quiescently atrophic but is active metabolically. Studies measuring uptake of tritiated thymidine,203 32P-radiolabeled phosphate,204 and acridine orange205 suggest LS is significantly more active than unaffected skin. Flow cytometry reveals some LS lesions to have aneuploidy commensurate with actinic keratoses and keratoacanthomas.206 However, there are problems with studies supporting a neoplastic potential of LS. These include failure to appreciate the normally increased metabolic activity in genital versus nongenital skin and the possibility that mononuclear cell infiltrates may lead to false-positive results based on techniques that assess nuclear activity.207
    A plausible explanation for the association of LS with cancer involves infection with HPV.208,209 It has been proposed that LS provides the "fertile field" on which an oncogenic virus may cause dysplastic changes.210 HPV is associated with hyperplastic dystrophy142 but is not consistently found in vulvar squamous cell carcinoma associated with LS.102
    Many techniques have been suggested to detect more significant dystrophy and dysplasia in the examination of a patient with LS. Cytologic study by Papanicolau smears from the external genitalia was suggested200 but has been criticized as too insensitive.208,211 Acetowhitening in conjunction with colposcopy142,212 can be useful in finding areas of more hypertrophic dystrophy. Use of toluidine blue may increase the sensitivity.92,180,200,208 However, false-positive findings may occur when toluidine blue stains lymphocyte nuclei in the lichenoid infiltrate under a thinned epidermis and mimics a more dysplastic process.213 With or without adjunctive techniques any ulcerated, friable, thickened, red, and nodular areas should be subjected to biopsy. However, the potential sampling error inherent with punch biopsies should be kept in mind.200,212
    Although squamous cell carcinoma is the primary malignancy associated with LS, other tumors have been rarely reported. These include basal cell epithelioma116,214 and melanoma.215 Despite a single report of several basal cell epitheliomas arising in extensive LS of the shoulders,216 it is generally accepted that cancer does not occur in extragenital or pediatric LS.217

    In 1910 Ormsby cautioned not to confuse LS with scleroderma218 and set the stage for what would be decades of debate regarding their association. Many authors have described coexistent LS and morphea, at all ages and in both sexes.134, 219-225 Morphea and LS coexisting in the same biospy specimen has likewise been described.139, 226,227 Although most reports describe extragenital LS associated with morphea, there is mention of genital LS and extragenital morphea.105,228 Wallace21 reported 13 patients with morphea in his series of 380 patients. The frequency of the reported association leads many to assume a relation between the two.72,229,230 With sequential biopsies, several investigators have reported transition from LS to morphea227 or vice versa.24 Borda et al.73 view LS as a scleroderma of the papillary dermis and classify LS as a subset of the greater body of scleroderma and "pseudo-scleroderma" diseases. They further resurrect the long-abandoned term "white spot scleroderma" as a transitional condition. Others view LS and morphea as manifestations of the same disease with morphea having a greater association with systemic disease.231,232 The proposal that LS, morphea, and systemic scleroderma are borrelial disease has received much recent attention.233 Acrodermatitis chronica atrophicans (ACA), well established as a spirochetal disease, is often considered the bridge between LS and morphea because of the degree of clinical and histologic overlap it shows.234-237
    However, many investigators believe there are enough clinical and histologic differences between LS and morphea to argue they are distinct if not unrelated diseases,  and that coexistent lesions are coincidental.45, 148,150,238,239 Morphea does not have the immunoreactant deposition at the dermoepidermal junction and papillary dermis that LS often does.240 Different lectin staining patterns have also been reported; only LS stains with Eulex eropaeus.241 The loss of elastic fibers characteristic of LS is not found in morphea151; beyond the differences in gross histologic features, changes in the dermal ground substance and dermoepidermal junction are distinct.145 Collagen biosynthesis is generally increased in scleroderma and decreased in LS.242,243 Transforming growth factor-b is increased in scleroderma and not in active LS.244 Morphea and ACA have similar nerve degeneration patterns that are distinct from LS.161 Patterson and Ackerman245 raised the issue that many cases diagnosed clinically and histologically as LS are actually morphea when sclerosus in the reticular dermis and subcutis are appreciated. They classify any case with involvement beyond the papillary dermis as morphea, regardless of how consistent the more superficial histologic characteristics may be with LS. They further suggest that the lack of significant infiltrate and minimal vacuolar interface changes in many cases in their series support a diagnosis of morphea. Earlier, however, Ackerman139,140 noted that late sclerotic lesions of LS may have only sparse infiltrate and interface changes.

    Beginning in 1910, with the report of a patient with both LS and diabetes mellitus,218 attempts have been made to link LS with known or suspected autoimmune disorders or with the presence of autoantibodies.246,247 Vitiligo and alopecia areata have been reported in association with genital and extragenital LS in both sexes.19,21,43,248-253 Thyroid disease, especially Graves' disease,63,254,255 and both type I and type II diabetes are likewise reported in LS.48,87,105,147,224,256 Other conditions thought to be immune-mediated and associated with LS include lichen planus,19,222,257 eczema and atopic dermatitis,106,253 polymyalgia rheumatica,252 psoriasis,251 primary biliary cirrhosis,124, 258,259 fasciitis,220, 257 myositis,231, 260 lupus panniculitis,257 systemic lupus erythematosus,132 and achlorhydria, with or without pernicious anemia.251,261,262
    Various autoantibodies have been reported to be more common in patients with LS, including thyroid antimicrosomal, antigastric parietal cell, and antinuclear antibodies.250,255,263 Anti-adrenal cortex129 and anti-mitochondrial259,264 antibodies are less commonly detected. A large study of 350 female patients with LS revealed 21.5% with at least one autoimmune disease and 42% with at least one autoantibody.189 A comparable study of 25 men with LS revealed 20% with vitiligo or alopecia areata and 36% with autoantibodies (mostly antismooth muscle and antigastric parietal cell).250, 265,266 Direct immunofluorescence has demonstrated nonspecific deposition of IgM, C3, and fibrin at the dermoepidermal junction and papillary dermis.267,268 Perivascular IgG and IgA were found in 63% of foreskins removed for LS.114 Elastic fibers with irregular diameters are seen in LS and are also characteristic of connective tissue disease.154 Carli et al.269 recently characterized the inflammatory infiltrate in LS and demonstrated consistently increased numbers of Langerhans cells, regardless of the age of the lesion and amount of infiltrate.
    Even advocates of an association of autoantibodies and autoimmune diseases with LS note that disease severity may be unrelated to these factors250 and that autoimmune conditions often predate the appearance of LS. Therefore an exhaustive search for these diseases is not mandated in a patient with LS.270,271 Perhaps the most provocative association between LS and an autoimmune phenomenon is a description of "paraneoplastic LS" in the comments to a case report in 1973.272 Here Curth described patients with unspecified internal malignancies whose LS was alleviated with tumor resection and flared with tumor recurrence. This is analogous to paraneoplastic pemphigus and has not been reported since.
    Finally, LS has been reported in identical65,273 and nonidentical twins,274 a pair of young sisters,100 three adult sisters,248 mothers and daughters,11, 217 and a brother and a sister.38 However, no consistent autosomal or X-linked genetic pattern has been determined and haplotype studies provide conflicting results.44,275 HLA haplotypes reported to be associated with LS include HLA B-40,217,276 Aw-30, Aw-31,277 DR-5, DR-7, B-21,278 A29, B-44,279 and B-27.280

    Most early reports of LS suggested the victims were primarily women who were "hysterical," "excitable," or "highly nervous."218,281,282 As late as 1952 nervousness was reported as a risk factor for LS.120 In 1903 Johnston and Sherwell74 proposed that local interference with blood supply led to LS and this idea was echoed in 1993.69 In 1913 Irvine237 injected serum from a patient with "dermatitis atrophicans maculosa" (which by its description may have been either LS or ACA) into healthy skin and produced an isolated atrophic plaque. Recent investigators have shown a subpopulation of dermal fibroblasts that proliferate on incubation with serum from patients with scleroderma.283,284 The factors causing inflammation and fibrosis in LS and scleroderma may be immunologically related in the same way idiopathic scleroderma and chronic graft-versus-host disease share common clinical and histologic features.226
    The concept that some, if not all, cases of LS might be caused by an infectious disease is not new. Beyond the possibility that a chronic "garden variety" infection might generate the isomorphic phenomenon102,122 attempts to isolate the responsible organism have yielded conflicting results. Cantwell285,286 proposed that an L-form, or cell-wall-deficient, atypical mycobacterium such as Mycobacterium, fortuitum caused scleroderma and LS. "Large bodies," invariably acid fast with the Fite strain, were identified around deep dermal blood vessels, eccrine glands, nerves, and in the fat. These bodies were sometimes difficult to distinguish from lipofuchsin, melanin, and hemosiderin deposits, and there are no recent follow-up reports. Török et al.,95 in a electronmicroscopic study of LS in children published in 1975, noted tubular particles in endothelial cells and fibrocytes that resembled paramyxovirus-like inclusion bodies. Despite the conclusion that these were probably not of viral origin and instead represented aberrant cytoplasmic tubules, the idea of a slow virus generating an autoimmune reponse was advanced. Others have also proposed an unspecified viral cause for LS.172
    In a 1913 case report of LS, the comment was made that the concern in properly classifying this new entity was unfounded because it was just another manifestation of syphilis.281 Although syphilis has been reported in patients with LS,18,287 a new spirochete is the center of interest. In addition to the research on the relation of Borrelia burgdorferi to scleroderma are many reports linking LS to this spirochete. ACA, once thought a form of scleroderma, and at times coexistent with LS,157,256 is now well established as a borrelial disease.234,288 Researchers have found spirochetes in the papillary dermis of as many as 48% of patients with LS, especially in early cases.289 Six of 13 patients with predominantly genital LS has spirochetes revealed by immunoperoxidase methods, inclduing a possible organism in a focus of basilar degeneration.290 Two patients with neuroborreliosis had both localized and generalized LS.233 Another patient with ACA and genital LS had spirochetes recovered from his urine.256
    Tuffanelli291 noted that false-positive borrelial immunofluorescence tests may occur because of coexistent connective tissue diseases and other spirochetal infections. Recognizing the problems with identifying spirochetes on special stains, a recent study employed the lymphocyte proliferative assay, a method of detecting prior T-cell sensitization to a specific antigen, and found their two patients with LS had both elevated borrelial antibodies and high lymphoproliferative responses.232 Acceptance of a borrelial cause for LS is far from universal and there are several negative studies.150,242 It would be difficult to explain all LS, especially the number of genital cases and those in very young children, on the basis of arthropod vector. Better modles for a spirochetal cause of LS might be those of endemic syphilis (bejel) or pinta. These spirochetal diseases are spread nonvenereally and affect even young children. In the case of pinta, the disease is confined to the skin.292
    Independent of autoantibodies, a possible mechanism of spirochete-induced fibrosis was revealed by the observation that killed spirochetes induced a macrophage culture to produce interleukin 1 (IL-1).293 Fibroblasts, under the influence of IL-1, proliferate and increase secretion of collagenase and prostaglandin E2. The continued production of IL-1 and other cytokines by Borrelia-activated T cells could maintain fibroblast stimulation resulting in the fibrosis in scleroderma and late LS.232 It is thought this effect of spirochetal antigens explains the occurrence of disease without identifiable spirochetes. Spirochetes are not the only source of fibrosis-stimulating antigens. A cellular immune reponse to collagen itself was suggested by one scleroderma study,294 and a collagen-induced migration inhibition factor has been studied in fibrotic lung disease.295 Pneumococcal polysaccharides in the presence of serum can also induce DNA synthesis in fibroblasts in the absence of macrophages.296 In scleroderma, fibroblasts were found to secrete procollagen types I and III in a normal ratio but at a rate twice that of control cells.297
    Although collagen changes in LS are often analagous to the changes in scleroderma, they exhibit some important differences. Some report decresed collagen synthesis, both type I and type III, in LS.138,242,298 Others report a slight increase in the synthesis of a younger, more soluble type I collagen244 or evidence of increased turnover and intracellular degradation without a significant net gain or loss.298 Still others have detected increased ground substance and evidence of collagen degeneration and regeneration with fewer normal fibers.146 Increased activity of amidase, an elastase-type protease, which is able to degrade human skin elastin, may cause the elastic tissue destruction in LS.146, 299 Retinoids down-regulate fibroblast proliferation and synthesis, which explains the utility of reinoids in treating LS.300
    Suggested by frequent local recurrence after vulvectomy, the contribution of local factors to the development and presistence of LS is well recognized. Some attribute this to continued factors of friction from opposed skin surfaces,125,137 chronic infection,117,119 irritation from glycosuria or other sources of chronic irritation.119 However, such factors do not apply to all cases of genital LS and few cases of extragenital LS. More provocative regarding the relevance of local factors are reports of recurrent LS of the scalp several years after excision and grafting,63 vulvar LS reappearing in a myocutaneous graft from the thigh,116 and an oft-quoted observation by Whimster,115 who reported that normal skin grafted to an involved vulva developed LS, whereas a graft of LS-involved skin applied to the donor site became normal in texture and appearance.
    The interaction of hormones, fibroblasts, local changes in collagen, cytokines, and other growth factors have been studied for several decades without universal agreement on the pathogenesis of LS. Hormonal studies in LS center on the actions of testosterone. Androgen-sensitive fibroblasts in genital skin facilitate collagen and glycosaminoglycan production.301,302 The increased testosterone metabolism in genital skin, which occurs at menarche, is believed to cause the spontaneous improvement that often occurs in childhood vulvar LS.303 In fact, adults with LS were found to have decreased serum levels of free testosterone, androstenedione, and fihydrotestosterone compared with control subjects. A defect in the function of 5a-reductase, an enzyme usually showing increased activity in genital skin compared with nongenital skin,302, 304,305 is believed to be the underlying defect predisposing these patients to LS.303 A study of risk factors for LS revealed that nonsmokers were significantly more likely to have LS than smokers. The authors conjecture that this is caused by the androgen-elevating effect of cigarette smoking.306

        LS was initially treated with a variety of irritating and keratolytic agents knowns as "revulsives." This resulted in inflammation and tissue damage with anticipated clinical improvement. Such agents include salicylic acid, trichloroacetic acid, resorcin,65, 67,74,281,287,307 thymol,50,84,308 boric acid ointment,309 carbolic acid,93 and carbol fuchsin.94 Physically destructive methods include suction cups,281 cryotherapy,21, 94,197,208 fulguration and desiccation,94,208,310 and tangential excision81 or dermabrasion.18 Carbon dioxide laser is the "modern revulsive." Used now primarily for treatment of hyperplastic vulvar dystrophies,208,210,212 it has been applied to both vulvar102,311 and penile LS312,313 with some benefit.
    In the first half of the century genital and extragenital LS was treated with various forms of radiation from ultrasound260 to infrared84 to Grenz rays.50 More aggressive radiotherapy with topically applied thorium X94,122 or "hard" x-rays41,53,80,93,185,218,314,315 persisted into the 1940s, even in children. Eventually, various authors pointed out how temporary117,184 the benefits were and how "evil radiation therapy"188 added clinical and histologic confusion as well as cancer risk to an otherwise benign process. The contribution of radiation therapy to the incidence of squamous cell carcinoma reported with LS in the earlier literature is uncertain. It is notable that most studies of the era report large numbers of patients so treated before the diagnosis of squamous cell carcinoma.183-185
    Bismuth (topically,47 orally,47, 84 and parenterally308, 316,317) was extensively used into the 1960s. Parenteral and oral mercury was likewise popular at one time.49, 247,281,307 An unusual, relatively recent use of mercury involved tattooing the vulva with mercuric sulfide for intractable vulvar pruritis.318 Intravenous procaine94, 319 and topical cocaine93 have also been used for persistent pruritis. However, allergic sensitization to topical anesthetics has been reported.94 Injection of alcohol into LS lesions320-322 or systemically in a gridlike fashion92 is still mentioned for relief of pruritis in recent texts on vulvar diseases. Antimalarials, orally47, 228,231,257,323,324 and intralesionally,325 have been reported beneficial in both penile and vulvar LS and have been employed in all age groups. Intralesional hyaluronidase was reported to be of no benefit.18 Potassium para-aminobenzoate (4 to 24 gm/day)326 is reported to be effective, possibly by decreasing mucopolysaccharide production.82 However, one study suggested it is not effective in men before circumcision.48 Chelation therapy231, 260 and topical crotamiton (often in conjunction wiht a topical steroid)179,180,327 have been used in hopes of remission or relief of associated pruritis. Topical80 and systemic antihistamines,190,231 cool milk compresses, and application of vegetable shortening are also recommended for the pruritis of LS.328 One of the more dramatic surgical options for persistent vulvar pruritis, short of vulvectomy, is the "Mering procedure" that entails cutting the entire labial and pubic region to denervate the vulva.329,330
    Surgery might still be the primary therapy for LS were it not for the introduction of hormonal therapy with corpus luteal extract injections in 1922.93 Parenteral adrenocorticotropic hormone (ACTH)81,83 is a relatively recent innovation, whereas topical,217,248,309 intraurethral331 or intralesional88, 111,165,325,332 hydrocortisone or triamcinolone21,323,333-335 have been the mainstays of genital102 and extragenital LS therapy.260,316 Oral corticosteroids are likewise effective but generally reserved for patients with other indications for their use.222 Combining topical steroids with other modalities such as keratolytics,67 pramoxine,180, 328 or occlusive therapy323 has additional benefits. Marked benefit has been reported from brief courses (usually 2 weeks or less) of topical fluorinated steroids102,254,327,335 or clobetasol.54 Such regimens are used even in children,56,96,101,103 and histologic regression of LS54 may occur as well as remarkable relief of symptoms. A new approach to the nonsurgical treatment of phimosis is Jørgenssen and Swenson's 1993 report173 in which clobetasol and gentle retraction were employed daily for up to 3 months and obviated the need for surgery in 70% of their patients. In this noncontrolled study, all patients failing to respond to topical therapy who underwent histologic examination of the foreskin were found to have LS.
    Topical estrogen has benefitted both vulvar80,160,316 and penile77 LS, although some believe the results leave much to be desired. Intralesional estrogen was used in a case of penile LS336 as were oral conjugated estrogens260; both provided some symptomatic relief. Topically applied testosterone, 2% to 2.5%87, 74,337 in petrolatum or paraffin,102 has not only demonstrated clinical efficacy but also histologic regression,254,338-340although Cinberg341 in his description of the first documented use of topical testosterone found minimal histologic regression despite significant clinical improvement. Reversal of abnormal touidine blue uptake was noted in one study.160 Reported side effects include enlargement of the clitoris (5% to 50%),342 increased libido (up to 50%), acne, virilization, and menstrual irregularities.92,254 Increased serum levels of testosterone were not detected.253 Alternating testosterone with a topical corticosteroid is another strategy to minimize the side effects of either agent.92 Local irritation is common and may be minimized by reduction of the concentration of testosterone to as low as 0.5%143,343 or compounding with a cream base instead of an ointment.329 Another means of avoiding the androgenic effects of testosterone, especially desirable in children, is the use of topical progesterone (200 mg in 2 ounces of hydrophilic ointment).254,344,345 However, progesterone is generally not as effective in hyperplastic dystrophy and slower to relieve symptomatic LS than testosterone.117,121 Enhanced efficacy can be achieved by applying the progesterone in the morning and a topical corticosteroid, such as betamethasone ointment, at night.345
    Nutritional therapy has been used in the treatment of LS. Oral administration of dilute hydrochloric acid was recommended because of the association with achlorhydria.195 Protein supplements, chlorophyll,190 and wheat germ oil94, 122,346 were staples in early nutritional therapies. Various B vitamins, orally and parenterally, have been recommended including B1,308,347 B12,94,122 and "B complex."347 Vitamin E has been recommended as adjunctive therapy217 (orally, topically, and intramuscularly), although it was not believed to be especially effective.47, 348 Vitamin D has likewise been endorsed.80 Use of topical vitamin D analogues such as calcipotriene is not reported. One recommended concoctopn contained "vitamin F."58 Oral and parenteral vitamin A has been proposed; the ealier reports recommend nutritional supplements such as cod liver oil.47,83,195,308,316,349-351 This is based on likening the hyperkeratosis seen in LS to a vitamin A deficiency and noting that achlorhydria, reported ofetn in LS, impairs absorption of vitamin A.351 Intralesional vitamin A was advocated by one group.353 Although no recent literature strongly advocates nutritional therapy for LS, the echoes of vitamin A therapy continue in current research on the efficacy of retinoids. Etretinate has been employed in dosages of 0.37 per day343 to 1 mg/kg per day354 with measurable succcess even in patients who had recurrent disease after vulvectomy.355 Severely dysplastic and hyperkeratotic lesions are most likely to recur after therapy.343 Some report concomitant histologic improvement,51,355 whereas others have noted only a decrease in hyperkeratosis.356,357 More recently acitretin (30 mg daily) has been used, and although pruritis was relieved, a large placebo response in this study made its ultimate efficacy less defined.25 Of note, the subjective and objective grading scale used in this report may have applications in future studies.
    Careful attention to hygiene is recommended, and efforts to treat coexistent vaginal infections102,321 or balanitis are essential. Because of the possible role of spirochetes in some cases of LS and morphea, many recommend empirical treatment with antispirochetal antibiotics such as penicillin or doxycycline.184,232,233,291 The utility of this therapy in LS remains to be defined.

        The true incidence of LS is unknown. Significant underreporting is likely for many reasons. These include faliure to recognize the condition, misdiagnosis as a related or unrelated disease, frequently asymptomatic patients, and reluctance to seek care because of embarassment or fear of legal complications (pediatric cases). Accurate epidemiology of LS is further hampered by fragmentation of case in various specialties (dermatology, gynecology, urology, pediatrics, family practice), many using different terminology for the same entity.
    LS is an inflammatory condition caused by an incompletely defined response to a variety of mechanical and antigenic stimulu. These stimuli include, among others, trauma, spirochetal infection, and autoimmune diseases.
    Further definition of the role of spirochetal diseases in LS is pivotal in making treatment recommendations. If this is a major cause of LS, the mode of transmission should be elucidated. Is the transmission via an insect vector (Lyme disease), sexually (venereal syphilis), or through casual person-to-person contact (endemic syphilis and pinta)?
    LS and scleroderma, although clinically and histologically distinct entities that may coexist, share many pathogenic mechanisms. Perhaps they represent expressions of the same antigenic insult with LS being expressed in the papillary dermis and scleroderma in the reticular dermis and deeper. Differences in vascular adhesion molecuels, elastic network, and relative amounts of type I and III collagen may explain the differences between the two conditions. An unidentified genetic or environmental factor may determine which disease the patient acquires.
    True LS is not itself a premalignant condition, although other white lesions that stimulate it, such as hyperplastic dystrophy, may be. LS should not be treated with aggressive surgical modalities. Prior radiation therapy may have artificially increased historical rates of "malignant transformation." The contribution of human papillomavirus to current sporadic cases of LS-associated genital squamous cell carcinoma is unknown.
    Urologists, in contrast to gynecologists, continue to view LS as primarily a surgical problem. Circumcision is still the most widely used primary therapy for genital LS in uncircumcised men. Reports of medical therapy are anecdotal and generally limited to persistent or recurrent lesions in patients already circumcised.
    Medical therapies include retinoids and topical corticosteroids (including superpotent agents), testosterone, and progesterone. Other systemic and topical treatments have been used with variable success. Genital LS in children presents some special treatment concerns.


  1. Hallopeau H. Leçons cliniques sur les maladées cutanées et syphiliques. Union Med Can 1887; 43: 472.
  2. Weir RF. Icthyosis of the tongue and vulva. NY State J Med 1875; 246.
  3. Hallopeau H. Lichen plan scléreux. Ann Derm Syph (2nd series) 1889; 20: 447-9.
  4. Hallopeau H. Sur un nouveau cas de lichen plan atrophique. Ann Derm Syph (3rd series)1896; 27: 57-60.
  5. Hallopeau H. Sur un nouveau cas de lichen de Wilson scléreux. Ann Derm Syph (3rd series) 1898; 9: 358-9.
  6. Darier J. Leçons cliniques sur les maladées cutanées et syphiliques [Comment]. Union Med Can 1887; 43: 472.
  7. Darier J. Lichen plan scléreux. Ann Derm Syph 1892; 23: 833-7.
  8. Unna PG. Karenblattförmige sklerodermie. Lehrbuch der speziell path Anatomie (8 lief). Berlin: A Hirschwald, 1894: 112.
  9. Westberg F. Ein Fall von mit weissen flecken Einherhehender, bisher nicht bekannten Dermatose. Monatschr Prakt Dermatol 1901; 33: 355-61.
  10. Von Zombusch LR. Über Lichen albus, eine bisher unbeschrieben Erkrankung. Arch Dermatol Syph (Berlin) 1906; 82: 339.
  11. Montgomery FH, Ormsby OS. "White spot disease" (morphea guttata) and lichen planus sclerosus et atrophicus. J Cutan Dis 1907; 25: 1-16.
  12. Csillag J. Dermatitis lichenoides chronica atrophicans. Ikonographia Dermatol 1909; 4: 147.
  13. Breisky A. Über Kraurosis vulvae. Z Heilkr 1885; 6: 69.
  14. Stühmer A. Balanitis xerotica obliterans (post operationem) und ihre beziehungem zur Kraurosis glandis et preaeputii Penis. Arch Derm Syph (Berlin) 1928; 156: 613-23.
  15. Friedrich EG. Lichen sclerosus. J Reprod Med 1976; 17: 147-54.
  16. Friedrich EG. New nomenclature for vulvar disease. Obstet Gynecol 1976; 47: 122-4.
  17. Gahan E. Hallopeau's communications on lichen sclerosus et atrophicus. AMA Arch Dermatol Syphilol 1954; 69: 435-7.
  18. Miller RF. Lichen sclerosus et atrophicus with oral involvement. AMA Arch Dermatol 1957; 43-55.
  19. Herzberg JJ, Meyer-Rohr J, Unna PJ. Sclerolichen Gougerot. Arch Klin Exp Dermatol 1963; 216: 246-59.
  20. Brookes DV, Sarkany I. Lichen sclerosus et atrophicus with mouth lesions. Br J Dermatol 1970; 83: 422.
  21. Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Derm Soc 1971; 57: 9-30.
  22. Siar CH, Ng KH. Oral lichen sclerosus et atrophicus. J Oral Med 1985; 40: 148-50.
  23. de Arujo VC, Orsini SC, Marcucci G, et al. Lichen sclerosus et atrophicus. Oral Surg Oral Med Oral Pathol 1985; 60: 655-7.
  24. Dalziel K, Reynolds AJ, Holt PJA. Lichen sclerosus et atrophicus with ocular and maxillary complications. Br J Dermatol 1987; 116: 735-7.
  25. Bousema MT, Romppanen U, Geiger J-M, et al. Acitretin in the treatment of severe lichen sclerosus et atrophicus of the vulva: a double-blind, placebo-controlled study. J Am Acad Dermatol 1994; 30: 225-31.
  26. Ravits HG, Welsh AL. Lichen sclerosus et atrophicus of the mouth. AMA Arch Dermatol 1957; 76: 56-8.
  27. McCarty L. Circumscribed scleroderma [Comment]. Arch Dermatol Syphilil 1939; 39: 904-7.
  28. Ormsby OS, Mitchell JH. Lichen planus atrophicus et sclerosus and kraurosis vulvae. Arch Derm Syph 1922; 5: 786.
  29. Freeman C, Laymon CW. Balanitis xerotica obliterans. Arch Dermatol Syphilol 1941; 44: 547-61.
  30. Laymon CW, Freeman C. Relationship of balanitis xerotica obliterans to lichen sclerosus et atrophicus. Arch Dermatol Syphilol 1944; 49: 57-9.
  31. Ginsburg L. Circumscribed scleroderma or leukoplakia of the vuvla in a child aged eight years. Arch Dermatol Syph 1939; 39: 903.
  32. Aaronson LD, Baler G, Schiff BL. Lichen sclerosus et atrophicus occurring in childhood. Arch Dermatol 1962; 85: 746-7.
  33. Dogliotti M, Bentley-Phillips CB, Schmaman A. Lichen sclerosus et atrophicus in the Bantu. Br J Dermatol 1974; 91: 81-5.
  34. Patel RI, Reed WB. Annular atrophic plaques of the face and upper body. Cutis 1979; 24: 90-3.
  35. Tagami H. Eosinophilic infiltration in lichen sclerosus et atrophicus [Letter]. Arch Dermatol 1972; 105; 606-7.
  36. Bernstein JC, Robinson HM. Circumscribed scleroderma. Arch Dermatol Syphilol 1939; 39: 904-7.
  37. Jacyk WK, Isaac F. Lichen sclerosus et atrophicus in Nigerians. J Natl Med Assoc 1979; 71: 387-8.
  38. Ebert MH. A case for diagnosis. Arch Dermatol Syphilol 1929; 19: 144-5.
  39. Senear FE, Caro MR, Bucholz AM. Lichen sclerosus et atrophicus. Arch Dermatol Syphilol. 1948; 57: 754-5.
  40. Mowas CM, Guzzo CA. A 14-year-old girl with hyperkeratotic plaques. Dermatol Today 1994; 4: 2-5.
  41. Woodruff JD, Baens JS. Interpretation of atrophic and hydrothrophic alterations in the vulvar epithelium. Am J Obstet Gynecol 1963; 86: 713-23.
  42. Brownstein MH. Lichen sclerosus et atrophicus [Comment]. Arch Dermatol 1973; 108: 433.
  43. Helm KF, Gibson LE, Muller SA. Lichen sclerosus et atrophicus in children and young adults. Pediatr Dermatol 1991; 8: 97-101.
  44. Thomas RHM, Ridley CM, Sherwood F, et al. The lack of association of lichen sclerosus et atrophicus with HLA-A and B tissue antigens. Clin Exp Dermatol 1984; 9: 290-2.
  45. Steigleder GK, Raab WP. Lichen sclerosus et atrophicus. Arch Dermatol 1961; 84: 219-26.
  46. Montgomery H, Hill WR. Lichen sclerosus et atrophicus. Arch Dermatol 1940; 42: 755-79.
  47. Oberfield RA. Lichen sclerosus et atrophicus and kraurosis vulvae. Arch Dermatol 1962; 83: 144-53.
  48. Garcia-Bravo B, Sánchez-Pedreno P, Rodríguez-Pichardo A, et al. Lichen sclerosus et atrophicus. J Am Acad Dermatol 1988; 19: 482-5.
  49. Crissey JT, Osborne ED, Jordon JW. Lichen sclerosus et atrophicus in children. NY State J Med 1955; 55: 2912-5.
  50. Hyman AB, Falk HC. White lesions of the vulva. Obstet Gynecol 1958; 12: 407-13.
  51. Hart WR, Norris HJ, Helwig EB. Relation of lichen sclerosus et atrophicus of the vulva to development of carcinoma. Obstet Gynecol 1975; 45: 369-77.
  52. Loening-Baucke V. Lichen sclerosus et atrophicus in children. Am J Dis Child 1991; 145: 1058-61.
  53. Chernosky MM, Derbes VJ, Burks JW. Lichen sclerosus et atrophicus in children. Arch Dermatol 1957; 75: 647-52.
  54. Dalziel KL, Millard PR, Wojnarowska F. The treatment of vulval lichen sclerosus with a very potent topical steroids (clobetasol proprionate 0.05%) cream. Br J Dermatol 1991; 124: 461-4.
  55. Clark JS, Muller SA. Lichen sclerosus et atrophicus in children. Arch Dermatol 1967; 95: 476-82.
  56. Laude TA, Narayanaswamy G, Rajkumar S. Lichen sclerosus et atrophicus in an eleven year old girl. Cutis 1980; 26: 78-80.
  57. Chalmers RJG, Burton PA, Bennet RF. Lichen sclerosus et atrophicus. Arch Dermatol 1984; 120: 1025-7.
  58. Campus GV, Ena P, Scuderi N. Surgical treatment of balanitis xerotica obliterans. Plast Reconstr Surg 1984; 73: 652-7.
  59. Ledwig PA, Weigand DA. Late circumcision and lichen sclerosus et atrophicus of the penis. J Am Acad Dermatol 1989; 20: 211-4.
  60. Schinella RA, Miranda D. Posthitis xerotica obliterans in circumcision specimens. Urology 1974; 3: 348-51.
  61. Bainbridge DR, Whitaker RH, Shepheard BGF. Balanitis xerotica obliterans and urinary obstruction. Br J Urol 1971; 43: 487-91.
  62. Klenk-Pfeifer E. Lichen sclerosus et atrophicus facei bei 12 jährigem Mädchen. Z Hautkr 1974; 49: 841.
  63. Foulds IS. Lichen sclerosus et atrophicus of the scalp. Br J Dermatol 1980; 103: 197-200.
  64. Purres J, Krull EA. Lichen sclerosus et atrophicus involving the palms. Arch Dermatol 1971; 104: 68-9.
  65. Wüstner H, Gartmann H. Palmoplantarar und genitoanaler lichen sclerosus et atrophicus bei einiigen Zwilligen. Z Hautkr 1974; 49: 841-2.
  66. Tudino ME, Wong AK. Bullous lichen sclerosus et atrophicus on the palms and wrists. Cutis 1984; 33: 475-6.
  67. Petrozzi JW, Wood MG, Tisa V. Palmar-plantar lichen sclerosus et atrophicus. Arch Dermatol 1979; 115: 884.
  68. Hammar H. Plantar lesions of lichen sclerosus et atrophicus accompanied by erythermalgia. Acta Derm Venereol (Stockh) 1978; 58: 91-2.
  69. Weigand DA. Microscopic features of lichen sclerosus et atrophicus in acrochordons: A clue to the cause of lichen sclerosus et atrophicus? J Am Acad Dermatol 1993; 28: 751-4.
  70. Fabry J. Über einen fall von Kraurosis penis bei gleichzeitigem Bestehen von Weissflecken am Skrotum. Dermatol Wochnschr 1928; 86: 7.
  71. Mackee GM, Wise F. White spot disease. J Cutan Dis 1914; 32: 629-60.
  72. Laymon CW. Lichen sclerosus et atrophicus and related disorders. Arch Dermatol 1951; 64: 620-7.
  73. Borda JM, Abulafia J, Jaimovich L. Syndrome of circumscribed sclero-atrophies. Derm Ibero Lat Am 1968; 3: 179-202.
  74. Johnston JC, Sherwell S. White spot disease. J Cutan Dis 1903; 21: 302-7.
  75. Edwards NW, Arnold HL. Corymbiform lichen sclerosus et atrophicus. Arch Dermatol 1979; 115: 1035.
  76. Mopper C, Courville C, Hendren O. Lichen sclerosus et atrophicus. Arch Dermatol 1956; 73: 611-3.
  77. Barclay DL, Macey HB, Reed RJ. Lichen sclerosus et atrophicus of the vulva in children. Obstet Gynecol 1966; 27: 637-42.
  78. Kaufman RH, Friedrich EG, Gardner HC. Benign diseases of the vulva and vagina. Chicago: Year Book Medical Publishers, 1989: 302-23.
  79. Wallace HJ, Whimster IW. Vulval atrophy and leukoplakia. Br J Dermatol 1951; 63: 241-57.
  80. Kindler T. Lichen sclerosus et atrophicus in young subjects. Br J Dermatol 1953; 269-79.
  81. Klein LE, Cohen SR, Weinstein M. Bullous lichen sclerosus et atrophicus: treatment by tangential excision. J Am Acad Dermatol 1984; 10: 346-50.
  82. Penneys NS. Treatment of lichen sclerosus with potassium para-aminobenzoate. J Am Acad Dermatol 1984; 10: 1039-42.
  83. Di Silverio A, Serri F. Generalized bullous and haemorrhagic lichen sclerosus et atrophicus. Br J Dermatol 1975; 93: 215-7.
  84. Gottschalk HR, Copper ZK. Lichen sclerosus et atrophicus with bullous lesions and extensive involvement. Arch Dermatol Syph 1947; 55: 433-40.
  85. Apisarnthanarax P, Osment LS, Montes LF. Extensive lichen sclerosus et atrophicus in a 7-year-old boy. Arch Dermatol 1972; 106: 94-6.
  86. Templeton HJ. Localized scleroderma with bullae. Arch Dermatol Syph 1941; 43: 361-5.
  87. Pasieczny TAH. The treatment of balanitis xerotica obliterans with testosterone propionate ointment. Acta Derm Venereol (Stockh) 1977; 57: 275-7.
  88. Staff WG. Urethral involvement in balanitis xerotica obliterans. Br J Urol 1970; 47: 234-9.
  89. Chalmers RJG, Burton PA, Bennett R, et al. Lichen sclerosus et atrophicus: a distinctive and common cause of phimosis in boys. Br J Dermatol 1982; 107 (suppl): 29-30.
  90. Jänner M, Schütte B, Breitbart E. Lichen sclerosus et atrophicus penis bei präpubertaeren knaben. Z Hautkr 1978; 53: 923-8.
  91. Anderton Rl, Abele DC. Lichen sclerosus et atrophicus in a vaccination site [Letter]. Arch Dermatol 1976; 112: 1787.
  92. Friedrich EG. Vulvar disease. Philadelphia: WB Saunders, 1983: 71-4, 129-42.
  93. Taussig FJ. Chronic leukoplakic vulvitis followed by cancer. Surg Clin North Am 1922; 2: 1559-70.
  94. Barker LP, Gross P. Lichen sclerosus et atrophicus of the female genitalia. Arch Dermatol 1962; 85: 362-73.
  95. Török E, Örley J, Görácz G, et al. Lichen sclerosus et atrophicus in children. Mod Probl Paediatr 1975; 17: 262-71.
  96. Berth-Jones J, Graham-Brown RAC, Burns DA. Lichen sclerosus et atrophicus: a review of 15 cases in young girls. Clin Exp Dermatol 1991; 16: 14-7.
  97. Jenny C, Kirby P, Furquay D. Genital lichen sclerosus mistaken for child sexual abuse. Pediatrics 1981; 83: 597-9.
  98. Handfield-Jones SE, Hinde FRJ, Kennedy CT. Lichen sclerosus et atrophicus in children misdiagnosed as sexual abuse. Br Med J 1987; 294: 1404-5.
  99. Priestley BL, Bleehen SS. Lichen sclerosus et atrophicus in children misdiagnosed as sexual abuse [Letter]. Br Med J 1987; 295: 211.
  100. Davidson DC, Clarke MDB, Kean HB. Lichen sclerosus et atrophicus in children misdiagnosed as sexual abuse [Letter]. Br Med J 1987; 295: 211.
  101. Berth-Jones J, Graham-Brown EAC, Burns DA. Lichen sclerosus. Arch Dis Child 1989; 64: 1204-6.
  102. Ridley CM. Lichen sclerosus et atrophcius. Semin Dermatol 1989; 8: 54-63.
  103. Ridley CM. Genital lichen sclerosus (lichen sclerosus et atrophicus) in childhood and adolescence. J R Soc Med 1993; 86: 69-75.
  104. Priestley BL, Bleehen SS. Lichen sclerosus and sexual abuse. Arch Dis Child 1990; 65: 335.
  105. Helm KF, Gibson LE, Muller SA. Lichen sclerosus et atrophicus in children and young adults. Pediatr Dermatol 1991; 8: 97-101.
  106. Ditkowsky SP, Falk AB, Baker N, et al. Lichen sclerosus et atrophicus in childhood. Am J Dis Child 1956; 91: 52-4.
  107. Huffman JW. Vulvar disorders in premenarchal children. Clin Obstet Gynecol 1960; 31: 154-63.
  108. Sanchez NP, Mihm MC. Reactive and neoplastic epithelial alterations of the vuvla. J Am Acad Dermatol 1982; 6: 378-88.
  109. Madden JF. The balanitides. JAMA 1935; 105: 420-7.
  110. Thomas RHM, Ridley CM, Black MM. Clinical features and therapy of lichen sclerosus et atrophicus affecting males. Clin Exp Dermatol 1987; 12: 126-8.
  111. Weitzner S. Posthitis xerotica obliterans. Am J Dis Child 1972; 123: 68-9.
  112. Potter B. Balanitis xerotica obliterans manifesting on stump of amputated penis. Arch Dermatol 1959; 79: 473-6.
  113. Mckay DL, Fuqua F, Weinberg AG. Balanitis xerotica obliterans in children. J Urol 1975; 114: 773-5.
  114. Bale PM, Lochhead A, Martin HCO, et al. Balanitis xerotica obliterans in children. Pediatr Pathol 1987; 7: 617-27.
  115. Whimster IW. The natural history of skin malignancy. Br J Dermatol 1965; 77: 534-5.
  116. di Paola GR, Rueda-Leverone NG, Belardi MG. Lichen sclerosus of the vulva recurrent after myocutaneous graft. J Reprod Med 1982; 27: 666-8.
  117. Adair Fl, Davis ME, Schuitema DM. Atrophy of the vulva. JAMA 1950; 114: 296-303.
  118. Montgomery H. Lichen sclerosus et atrophicus [Comment]. Arch Dermatol Syph 1951; 64: 654-5.
  119. Jeffcoate TNA. Chronic vulvar dystrophies. Am J Obstet Gynecol 1966; 95: 61-74.
  120. Cockerell EG, Knox JM, Rogers SF. Lichen sclerosus et atrophicus. Obstet Gynecol 1960; 15: 554-9.
  121. Friedrich EG. Vulvar dystrophy. Clin Obstet Gynecol 1985; 28: 178-87.
  122. Barker LP, Gross P. Lichen sclerosus et atrophicus of the female genitalia. Arch Dermatol 1962; 85: 102-13.
  123. McAdams AJ, Kirstner RW. The relationship of chronic vulvar disease, leukoplakia, and carcinoma in situ to carcinoma of the vulva. Cancer 1958; 11: 740-57.
  124. Panet-Raymond G, Dirard C. Lichen sclerosus et atrophicus. Can Med J 1972; 106: 1332-4.
  125. Taylor H. Leukoplakia of the vulva [Comment]. Am J Obstet Gynecol 1952; 64: 778.
  126. Pock L. Koebner phenomenon in lichen sclerosus et atrophicus. Dermatologica 1990; 181: 76-7.
  127. Pass CJ. An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar. Cutis 1984; 33: 405-6.
  128. Rothman S. Lichen sclerosus et atrophicus. Arch Dermatol 1957; 75: 294.
  129. Yates VM, King CM, Dave VK. Lichen sclerosus et atrophicus following radiation therapy. Arch Dermatol 1985; 121: 1044-7.
  130. Milligan A, Graham-Brown RA, Burns DA. Lichen sclerosus et atrophicus following sunburn. Clin Exp Dermatol 1988; 13: 36-7.
  131. Meffert JJ, Grimwood RR. Lichen sclerosus et atrophicus appearing in an old burn scar. J Am Acad Dermatol 1994; 31: 671-3.
  132. Kahana M, Levy A, Schewach-Millet M, et al. Appearance of lupus erythematosus in a patient with lichen sclerosus et atrophicus of the elbows [Letter]. J Am Acad Dermatol 1985; 12: 127-9.
  133. Ronnen M, Suster S, Kahana M, et al. Bilateral Koebner phenomenon in lichen sclerosus et atrophicus. Int J Dermatol 1987; 26: 117-8.
  134. Gross P. Generalized lichen sclerosus et atrophicus associated with band-like scleroderma (Koebner phenomenon and lichen sclerosus). Arch Dermatol 1958; 77: 752-3.
  135. Schamberg JF, Hirschler R. Lichen planus sclerosus. JAMA 1909; 53: 369-73.
  136. Ormsby OS. Lichen planus atrophicus (Hallopeau). J Cutan Dis 1910; 28: 460.
  137. Taylor HC. Leukoplakia of the vulva [Comment]. Am J Obstet Gynecol 1947; 54: 560.
  138. Hewitt J. Histologic criteria for lichen sclerosus of the vulva. J Reprod Med 1986; 31: 781-7.
  139. Ackerman AB. Histologic diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger, 1978: 198-202, 760-5.
  140. Ackerman AB. The lives of lesions. Philadelphia: Lea & Febiger, 1984: 131-7, 194-201.
  141. Jablonska SI. Scleroderma and pseudoscleroderma. Warsaw: Panstwowy Zalkad Lekarskich, 1965: 265-76.
  142. Barbero M, Micheletti L, Borgno G, et al. Vulvar dystrophies in young and premenopausal women. J Reprod Med 1988; 33: 555-8.
  143. Friedrich EG, MacLaren NK. Genetic aspects of vulvar lichen sclerosus. Am J Obstet Gynecol 1984; 150: 161-6.
  144. Shelley WB, Levy EJ. Histologic observations on the human apocrine sweat gland in health and disease. J Invest Dermatol 1955; 25: 249-63.
  145. Stoughton R, Wells G. A histochemical study on polysaccharides in normal and diseased skin. J Invest Dermatol 1950; 14: 37-51.
  146. Frances C, Wechsler J, Meimon G, et al. Investigation of intercellular matrix macromolecules involved in lichen sclerosus. Acta Derm Venereol (Stockh) 1983; 63: 483-90.
  147. Suurmond D. Lichen sclerosus et atrophicus of the vulva. Arch Dermatol 1964; 90: 143-52.
  148. Ackerman AB, Niven J, Grant-Kels JM. Differential diagnosis in dermatopathology. Philadelphia: Lea & Fabiger, 1982: 62-5.
  149. Romppanen U, Rantala I, Lauslahti K, et al. Light and electron microscope findings in lichen sclerosus of the vulva during etretinate treatment. Dermatologica 1987; 175: 33-40.
  150. Rahbari H. Histochemical differentiation of localized morphea-scleroderma and lichen sclerosus et atrophicus. J Cutan Pathol 1989; 16: 342-7.
  151. Winer LH. Elastic fibers in unusual dermatoses. Arch Dermatol 1955; 71: 338-48.
  152. Ketron W, Ellis FA. Kraurosis vulvae and white spot scleroderma. Arch Dermatol Syph 1939; 39: 904.
  153. Mann PR, Cowan MA. Ultrastructural changes in four cases of lichen sclerosus et atrophicus. Br J Dermatol 1973; 89: 223-31.
  154. Daróczy J, Török E. Electronmicroscopy of lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc 1974; 60: 183-7.
  155. Forsman WG, Holzmann H, Cabre J. Elektronenmikroskopische Untersuchungen der Haut beim Lichen Sclerosus et Atrophicans. Arch Klin Exp Dermatol 1964; 220: 584-99.
  156. Sánchez E, Aguilar A, Requena L, et al. Nevus elasticus and lichen sclerosus et atrophicus on the vulva. Cutis 1990; 45: 252-5.
  157. Asbrink E, Hovmark A. Cutaneous manifestations in Ixodes-borne Borrelia spirochetosis. Int J Dermatol 1987; 26: 215-23.
  158. Ketron LW, Ellis FA. Kraurosis vulvae (leucoplacia) and scleroderma circumscripta. Surg Gynecol Obstet 1935; 61: 635-50.
  159. Janovski NA, Ames S. Lichen sclerosus et atrophicus of the vulva: a poorly understood disease entity. Obstet Gynecol 1963; 22: 697-708.
  160. Lascano EF, Montes LF, Mazzini MA. Lichen sclerosus et atrophicus in childhood. Obstet Gynecol 1964; 24: 872-7.
  161. Pawlowski A. The nerve network of the skin in diffuse scleroderma and clinically similar conditions. Arch Dermatol 1963; 88: 236-41.
  162. Leppard B, Sneddon IB. Milia occurring in lichen sclerosus et atrophicus. Br J Dermatol 1975; 92: 711-4.
  163. Garat JM, Chéchile G, Algaba F, et al. Balanitis xerotica obliterans in children. J urol 1986; 136: 436-7.
  164. Leifer W. Balanitis xerotica obliterans. Arch Dermatol Syph. 1944; 49: 118-9.
  165. Catterall RD, Oates JK. Treatment of balanitis xerotica obliterans with hydrocortisone injections. Br J Vener Dis 1962; 38: 75-7.
  166. Shapiro L. Lichen sclerosus et atrophicus: first reported case in a boy. Dermatologica 1968; 136: 155-9.
  167. Mikat DM, Ackerman HR, Mikat KW. Balanitis xerotica obliterans: report of a case in an 11-year-old and review of the literature. Pediatrics 1973; 52: 41-4.
  168. Clemmensen OJ, Krogh J, Petri M. The histologic  spectrum of prepuces from patients with phimosis. Am J Dermatopathol 1988; 10: 104-8.
  169. Gairdner D. The fate of the foreskin. Br Med J 1949; 2: 1433-7.
  170. Øster J. Further fate of the foreskin. Arch Dis Child 1968; 43: 200-3.
  171. Spicer RD. Genital lichen sclerosus et atrophicus. J R Soc Med 1993; 86: 556.
  172. Jørgensen ET, Svensson A. The treatment of phimosis in boys, with a potent topical steroid (clobetasol proprionate 0.05%) cream. Acta Derm Venereol (Stockh) 1993; 73: 55-6.
  173. Rickwood AMK, Hemalatha V, Batcup G, et al. Phimosis in boys. Br J Urol 1980; 50: 147-50.
  174. Pride HB, Miller OF III, Tyler WB. Penile squamous cell carcinoma arising from balanitis xerotica obliterans. J Am Acad Dermatol 1993; 29: 469-73.
  175. Bingham JS. Carcinoma of the penis developing lichen sclerosus et atrophicus. Br J vener Dis 1978; 54: 350-1.
  176. Weigand DA. Lichen sclerosus et atrophicus, multiple dysplastic keratoses and squamous cell carcinoma of the glans penis. J Dermatol Surg Oncol 1980; 6: 45-50.
  177. Bart RS, Kopf AW. Squamous cell carcinoma arising in balanitis xerotica obliterans. J Dermatol Surg Oncol 1978; 4: 556-8.
  178. Weber P, Rabinowitz H, Garland L. Verrucous carcinoma in penile lichen sclerosus et atrophicus. J Dermatol Surg Oncol 1978; 13: 529-32.
  179. Reid R, Greenberg MD, Daoud Y, et al. Colposcopic findings in women with vulvar pain syndromes. J Reprod Med 1988; 33: 523-32.
  180. Rodke G, Friedrich EF, Wilkinson EJ. Malignant potential of mixed vulvar dystrophy (lichen sclerosus associated with squamous cell hyperplasia). J Reprod Med 1988; 33: 545-50.
  181. Ridley CM, Frankman O, Jones ISC, et al. New nomenclature for vulvar disease. Am J Obstet Gynecol 1989; 160: 769.
  182. Gardner HL, Friedrich EG, Kaufman RH, et al. The vulvar dystrophies, atypias, and carcinomata in situ: an invitational symposium. J Reprod Med 1976; 17: 131-6.
  183. Taussig FJ. Precancerous lesions of the skin of the vulva. Arch Dermatol Syph 1920; 1: 621-35.
  184. Taussig FJ. Leukoplakic vulvitis and cancer of the vulva (etiology, histopathology, treatment, five-year results). Am J Obstet Gynecol 1929; 18: 472-503.
  185. Taussig FJ. Cancer of the vulva. Am J Obstet Gynecol 1940; 40: 764-79.
  186. Parks J. Similarity of mouth and vulvar lesions. Am J Obstet Gynecol 1945; 49: 396-400.
  187. Kaufman RH. Hyperplastic dystrophy. J Reprod Med 1976; 17: 137-45.
  188. Franks AG. Balanitis xerotica obliterans. J Urol 1946; 56: 243-5.
  189. Thomas RHM, Ridley CM, McGibbon DH, et al. Lichen sclerosus et atrophicus and autoimmunity: a study of 350 women. Br J Dermatol 1988; 118: 41-6.
  190. Miller NF, Parrott MH, Stryker J, et al. Leucoplakia of the vulva. Am J Obstet Gynecol 1947; 54: 543-60.
  191. Newbold PCH. Pre-cancer and the skin. Br J Dermatol 1972; 86: 417-34.
  192. Davis ME. The differential diagnosis and treatment of vulvar lesions. Surg Clin North Am 1950; 30: 267-86.
  193. Graves WP, Smith GVS. Kraurosis vulvae. JAMA 1929; 92: 1244-52.
  194. Healy WP. Leukoplakia of the vulva [Comment]. Am J Obstet Gynecol 1947; 54: 559.
  195. Langley II, Hertig AT, Smith GVS. Relation of leucoplakic vulvitis to squamous cell carcinoma of the vulva. Am J Obstet Gynecol 1951; 62: 167-9.
  196. Camacho-Martinez F, navarro J, Moreno JC, et al. International dermatosurgery: reconstructive treatment of the vulva—a new procedure. J Dermatol Surg Oncol 1987; 13: 488-94.
  197. August PJ, Milward TM. Cryosurgery in the treatment of lichen sclerosus et atrophicus of the vulva. Br J Dermatol 1980; 103: 667-70.
  198. Cario GM. House MJ, Paradinas FJ. Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy in an 18-year0old girl. Br J Obstet Gynaecol 1984; 91: 87-90.
  199. Jeffcoate TNA, Woodcok AS. Premalignant conditions of the vulva, with particular reference to chronic epithelial dystrophies. Br Med J 1961; 2: 127-34.
  200. Woodruff JD. Vulvar atypia and carcinoma in situ. J Reprod Med 1976; 17: 155-63.
  201. Kaufman RH, Gardner HL, Brown D, et al. Vulvar dystrophies: an evaluation. Am J Obstet Gynecol 1974; 120: 363-7.
  202. Cockerell EG, Knox JM, Rogers SF. Lichen sclerosus et atrophicus. Obstet Gynecol 1960; 15: 554-9.
  203. Woodruff JD, Borkowf HI, Holzman GB, et al. Metabolic activity in normal and abnormal vulvar epithelia. Am J Obstet Gynecol 1965; 91: 809-19.
  204. Clark DGC, Zumoff B, Brunschwig A, et al. Preferential uptake of phosphate by premalignant and malignant lesions of the vulva. Cancer 1960; 13: 775-9.
  205. Friedrich EG, Julian CG, Woodruff JD. Acridine orange fluorescence in vulvar dysplasia. Am J Obstet Gynecol 1964; 90: 1281-7.
  206. Newton JA, Camplejohn RS, McGibbin DH. A flow cytometric study of the significance of DNA aneuploidy in cutaneous lesions. Br J Dermatol 1987; 117: 169-74.
  207. Kaufman RH, Gardner HL, Johnson PC. P32uptake in lichen sclerosus et atrophicus of the vulva. Am J Obstet Gynecol 1967; 98: 312-9.
  208. Woodruff JD. Carcinoma in situ of the vulva. Clin Obstet Gynecol 1985; 28: 230-9.
  209. Buscema J, Stern J, Woodruff JD. The significance of the histologic alterations adjacent to invasive vulvar carcinoma. Am J Obstet Gynecol 1980; 137: 902-9.
  210. Reid P. Physical and surgical principles governing expertise with the carbon dioxide laser. Obstet Gynecol Clin North Am 1987; 14: 513-35.
  211. Soper JT, Creaseman WT. Vulvar dystrophies. Clin Obstet Gynecol 1986; 29: 431-9.
  212. Reid R, Elfont EA, Zirkin EM, et al. Superficial laser vulvectomy. Am J Obstet Gynecol 1985; 152: 261-71.
  213. Collins CG, Hansen LH, Theriot E. A clnical stain for use in selecting biopsy sites in patients with vulvar disease. Obstet Gynecol 1966; 28: 158-63.
  214. Thomas RHM, McGibbon, Munro DD. Basal cell carcinoma of the vulva in associaiton with vulval lichen sclerosus et atrophicus. J R Soc Med 1985; 78 (suppl 11): 16-8.
  215. Friedman RJ, Kopf AW, Jones WB. Malignant melanoma in association with lichen sclerosus on the vulva of a 14-year-old. Am J Dermatopathol 1984; 6 (suppl 1): 253-6.
  216. Wise F, Shelmire B. Chronic atrophic lichenoid dermatitis (Csillag). Arch Dermatol Syph 1928; 18: 179-99.
  217. Shirer JA, Ray MC. Familial occurrence of lichen sclerosus et atrophicus. Arch Dermatol 1987; 123: 485-8.
  218. Ormsby OS. Lichen planus sclerosus et atrophicus (Hallopeau). JAMA 1910; 55: 901-6.
  219. Miller JL. Scleroderma en bande and lichen sclerosus et atrophicus. Arch Dermatol 1957; 76: 144-5.
  220. Mensing H, Schmidt KU. Diffuse fasciitis with eosinophilia associated with morphea and lichen sclerosus et atrophicus. Acta Derm Venereol (Stockh) 1985; 65: 80-3.
  221. Tafelkruyer J, Claessens FLE. Lichen sclerosus et atrophicus and scleroderma circumscripta. Dermatologica 1978; 156: 313-6.
  222. Connelly MG, Winkelmann RK. Coexistence of lichen sclerosus, morphea, and lichen planus. J Am Acad Dermatol 1985; 12: 844-51.
  223. Thomas RHM, Ridley CM, Black MM. Lichen sclerosus et atrophicus associated with systemic lupus erythematosus [Letter]. J Am Acad Dermatol 1985; 13: 832.
  224. Tremaine R, Adam JE, Orizaga M. Morphea coexisting with lichen sclerosus et atrophicus. Int J Dermatol 1990; 29: 486-9.
  225. Hauser W. Zur nosologischen Stellung des Lichen Sclerosus et Atrophicus. Arch Klin Exp Dermatol 1958; 208: 44-52.
  226. Natarajan S, Green ST. Generalized morphoea, lichen sclerosus et atrophicus, and primary biliary cirrhosis. Clin Exp Dermatol 1986; 11: 304-8.
  227. Uitto J, Santa Cruz DJ, Bauer EA, et al. Morphea and lichen sclerosus et atrophicus. J Am Acad Dermatol 1980; 3: 271-9.
  228. Curth W, Curth HO. Diagnosis: generalized scleroderma or lichen sclerosus et atrophicus. Arch Dermatol 1962; 85: 117-9.
  229. Witten VH, Gelfarb M. Scleroderma: guttate morphea vs. lichen sclerosus et atrophicus. Arch Dermatol 1962; 85: 546-8.
  230. Pierard GE, Arrese-Estrada J, Pierard-Franchimont C, et al. Is there a link between dendrocytes, fibrosis, and sclerosis? Dermatologica 1990; 181: 264-5.
  231. Lewis GM. Scleroderma: Lichen sclerosus et atrophicus? Arch Dermatol 1961; 84: 146-8.
  232. Buechner SA, Winkelmann RK, Lautenschlager S, et al. Localized scleroderma assocaited with Borrelia burgdorferi infection. J Am Acad Dermatol 1993; 29: 190-6.
  233. Aberer E, Kollegger H, Kristoferitsch W, et al. Neuroborreliosis in morphea and lichen sclerosus et atrophicus. J Am Acad Dermatol 1988; 19: 820-5.
  234. Abele DC, Anders KH. The many faces of phases of borreliosis II. J Am Acad Dermatol 1990; 23: 401-10.
  235. Montgomery H, Sullivan RR. Acrodermatitis atrophicans chronica. Arch Dermatol Syph 1945; 51: 32-47.
  236. Asbrink E, Brehmer-Andersson E, Hovmark A. Acrodermatitis chronica atrophicans: a spirochetosis. Am J Dermatopathol 1986; 8: 209-19.
  237. Irvine HG. Idiopathic atrophy of the skin. JAMA 1913; 61: 396-400.
  238. Senear FE. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1948; 58: 540.
  239. Goodman MH. Circumscribed scleroderma [Comment]. Arch Dermatol Syph 1939; 39: 904-7.
  240. Chorzelski T, Jablonska S. Coexistence of lupus erythematosus and scleroderma in light of immunopathological investigations. Acta Derm Venereol (Stockh) 1970; 50: 81-5.
  241. Shono S, Imura M, Ota M, et al. Lichen sclerosus et atrophicus, morphea, and coexistence of both diseases. Arch Dermatol 1991; 127: 1352-6.
  242. Panizzon R, Vuorio T, Bruckner-Tuderman L. Collagen biosynthesis and type I and type III procollagen mRNA in lichen sclerosus et atrophicus. Arch Dermatol Res 1990; 282: 480-3.
  243. Goltz R, Pinkus H, Winklemann RK, et al. Questions to the editorial board and other authorities. Am J Dermatolpathol 1980; 2: 283-6.
  244. Oikarinen A, Sandberg M, Hurskainen T, et al. Collagen biosynthesis in lichen sclerosus et atrophicus studied by biochemical and in situ hybridization techniques. Acta Derm Venereol (Stockh) 1991; 16: 3-12.
  245. Patterson JAK, Ackerman AB. Lichen sclerosus et atrophicus is not related to morphea. Am J Dermatopathol 1984; 6: 323-35.
  246. Tremaine RDL, Miller RA. Lichen sclerosus et atrophicus. Int J Dermatol 1989; 28: 10-6.
  247. Nomland R. Lichen sclerosus et atrophicus (Hallopeau) and related cutaneous atrophies. Arch Dermatol Syph 1930; 21P: 575-94.
  248. Murphy FR, Lipa M, Haberman HF. Familial vulvar dystrophy of lichen sclerosus type. Arch Dermatol 1982; 118: 329-31.
  249. Thomas RHM, Ridley CM, Black MM. The association of lichen sclerosus et atrophicus and autoimmune-related disease in males. Br J Dermatol 1983; 109: 661-4.
  250. Goolamali SK, Barnes EW, Irvine WJ, et al. Organ-specific antibodies in patients with lichen sclerosus. Br Med J 1974; 4: 78-9.
  251. Cunliffe WJ, Newell DJ, Hall R, et al. Vitiligo, thyroid disease, and autoimmunity. Br J Dermatol 1968; 80: 135-9.
  252. Faergemann J. Lichen sclerosus et atrophicus generalisata, alopecia areata, and polymyalgia rheumatica found in the same patient. Cutis 1979; 23: 757-8.
  253. Williams GA, Richardson AC, Hatchcock EW. Topical testosterone in dystrophic diseases of the vulva. Am J Obstet Gynecol 1966; 96: 21-30.
  254. Ayhan A, Urman B, Yuece K, et al. Topical testotosterone for lichen sclerosus. Int J Gynaecol Obstet 1989; 30: 253-5.
  255. Poskitt L, Wojnarowska F. Lichen sclerosus as a cutaneous manifestation of thyroid disease [Letter]. J Am Acad Dermatol 1993; 28: 665.
  256. Aberer E, Neumann R, Lubec G. Acrodermatitis chronica atrophicans in association with lichen sclerosus et atrophicans: tubulo-intersititial nephritis and urinary excretion of spirochete-like organisms. Acta Derm Venereol (Stockh) 1987; 67: 62-5.
  257. Winkelmann RK, Connolly Sm, Doyle JA. Carpal tunnel syndrome in cutaneous connective tissue disease: generalized morphea, lichen sclerosus, fasciitis, discoid lupus erythematosus, and lupus panniculitus. J Am Acad Dermatol 1982; 7: 94-9.
  258. Graham-Brown RAC, Sarkany I. Lichen sclerosus et atrophicus. Int J Dermatol 1986; 25: 317.
  259. Lavery HA, Pinkerton JHM, Callender M. The association of lichen sclerosus et atrophicus and primary biliary cirrhosis [Letter]. Br J Dermatol 1985; 112: 729-30.
  260. Lynch FW, Montgomery H, Brunstig LA. Morphea, lichen sclerosus et atrophicus, alopeacia areata, vitiligo. Arch Dermatol 1962; 85: 681-2.
  261. Sloan PJM, Goepel J. Lichen sclerosus et atrophicus and perianal carcinoma: a case report. Clin Exp Dermatol 1981; 6: 399-402.
  262. Bor S, Feiwel M, Chanarin I. Vitiligo and its aetiological relationship to organi-specific autoimmune disease. Br J Dermatol 1969; 81: 83-8.
  263. Harrington CI, Dunsmore IR. An investigation into the incidence of auto-immune disorders in patients with lichen sclerosus and atrophicus. Br J Dermatol 1981; 104: 653-6.
  264. Lavery HA, Pinkerton JHM, Callendar M. Association between lichen sclerosus et atrophicus and primary biliary cirrhosis [Letter]. Br J Dermatol 1986; 114: 515.
  265. Thomas RHM, Ridley CM, Black MM. The association between lichen sclerosus et atrophicus and autoimmune disease in males: an addendum [Letter]. Br J Dermatol 1984; 111: 371-2.
  266. Thomas RHM, Ridley CM, Black MM. The association between lichen sclerosus et atrophicus and auto-immune diseases in males is not proven [Letter]. Br J Dermatol 1984; 111: 122.
  267. Bushkell LL, Friedrich EG, Jordon RE. An appraisal of routine direct immunofluorescence in vulvar disorders. Acta Derm Venereol (Stockh) 1981; 61: 157-61.
  268. Dickie RJ, Horne CHW, Sutherland HW, et al. Direct evidence of localised immunological damage in vulvar lichen sclerosus et atrophicus [Letter]. J Clin Pathol 1982; 35: 1395-7.
  269. Carli P, Cattaneo A, Pimpinelli N, et al. Immunohistochemical evidence of skin immune system involvement in vulvar lichen sclerosus et atrophicus. Dermatologica 1991; 182: 18-22.
  270. Thomas RHM, Holmes RC, Rowland Payne CME, et al. The incidence of development of autoimmune diseases in women after diagnosis of lichen sclerosus et atrophicus [Abstract]. Br J Dermatol 1982; 107 (suppl 22): 29.
  271. Kahana M, Levy A, Schewach-Millet M, et al. Lichen sclerosus et atrophicus assocaited with systemic lupus erythematosus [Letter]. J Am Acad Dermatol 1985; 13: 832-3.
  272. Curth HO. Lichen sclerosus et atrophicus [Comment]. Arch Dermatol 1973; 108: 433.
  273. Thomas RHM, Kennedy CT. The development of lichen sclerosus et atrophicus in monozygotic twin girls. Br J Dermatol 1986; 114: 277-9.
  274. Cox NH, Mitchell JNS, Morley Wn. Lichen sclerosus et atrophicus in non-identical female twins [Letter]. Br J Dermatol 1986; 115: 743-6.
  275. Holt PJA, Darke C. HLA antigens in lichen sclerosus et atrophicus [Letter]. Clin Exp Dermatol 1985; 10: 182-3.
  276. Harrington CI, Gelsthorpe K. The association between lichen sclerosus et atrophicus and HLA-B40. Br J Dermatol 1981; 104: 561-2.
  277. Holt PJA, Darke C. HLA antigens and Bf allotypes in lichen sclerosus et atrophicus. Tissue Antigens 1983; 22: 89-91.
  278. Sideri M, Rognoni M, Rizzolo L, et al. Antigens of the HLA system in women with vulvar lichen sclerosus. J Reprod Med 1988; 33: 551-3.
  279. Purcell KG, Spencer LV, Simpson PM, et al. HLA antigens in lichen sclerosus et atrophicus. Arch Dermatol 1990; 126: 1043-5.
  280. Daunt SO, Kotowski KE, Reilly AP, et al. Ulcerative vulvitis in Reiter's syndrome. Br J Vener Dis 1982; 58: 405-7.
  281. Hazen HH. An anomalous case of white-spot disease. JAMA 1913; 61: 393-6.
  282. Brain RT. Lichen sclerosus et atrophicus. Br J Dermatol 1950; 62: 449-51.
  283. Needleman BW, Ordonex JV, Taramelli D, et al. In vitro identification of a subpopulation of fibroblasts that produces high levels of collagen in scleroderma patients. Arthritis Rheum 1990; 33: 842-52.
  284. Botsetin GR, Scherer GK, Leroy EC. Fibroblast selection in scleroderma. Arthritis Rheum 1982; 25: 189-95.
  285. Cantwell AR. Histologic forms resembling "large bodies" in scleroderma and "pseudoscleroderma." Am J Dermatopathol 1980; 2: 272-6.
  286. Cantwell AR. Histologic observations of pleomorphic, variably acid-fast bacteria in scleroderma, morphea, and lichen sclerosus et atrophicus. Int J Dermatol 1984; 23: 45-52.
  287. Sherwell S. A case of white spot disease. J Cutan Dis 1904; 22: 188-9.
  288. Stanek G, Wewalka G, Groh V, et al. Differences between Lyme disease and European arthropod-borne Borrelia infections. Lancet 1985; 1: 401.
  289. Ross SA, Sánchez JL, Taboas JO. Spirochetal forms in the dermal lesions of morphea and lichen sclerosus et atrophicus. Am J Dermatopathol 1990; 12: 357-62.
  290. Aberer E, Stanek G. Histological evidence for spirochetal origin of morphea and lichen sclerosus atrophicans. Am J Dermatopathol 1987; 9: 374-9.
  291. Tuffanelli D. Do some patients with morphea and lichen sclerosus et atrophicans have a borrelia infection? Am J Dermatopathol 1987; 9: 371-3.
  292. Koff AB, Rosen T. Nonvenereal terponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol 1993; 29: 519-35.
  293. Habicht GS, Beck ZG, Benach JL, et al. Lyme disease spirochetes induce human and murine interleukin 1 production. J Immunol 1985; 134: 147-54.
  294. Stuart JM, Postelwaite AE, Kang AH. Evidence for cell-mediated immunity to collagen in progressive systemic sclerosis. J Lab Clin Med 1976; 88: 601-7.
  295. Kravis TC, Ahmed A, Brown TE, et al. Pathogenic mechanisms in pulmonary fibrosis. J Clin Invest 1976; 58: 1223-32.
  296. Persson U, Möller G. Effect of polyclonal B-cell activators on DNA synthesis in fibroblasts. Scand J Immunol 1965; 4: 527-34.
  297. Uitto J, Bauer EA, Eisen AZ. Increased biosynthesis of triple-helical type I and type III procollagens associated with unaltered expression of collagenase by skin fibroblasts in culture. J Clin Invest 1979; 64: 921-30.
  298. Panizzon R, Tuderman L, Vuorio T. Decreased collagen biosynthesis and unchanged procollagen-mRNA type I and type III levels in fibroblasts from lesional foreskin of 3 patients with lichen sclerosus et atrophicus [Abstract]. J Invest Dermatol 1986; 87: 161.
  299. Godeau G, Frances C, Hornebeck W, et al. Isolation and partial characterization of an elastase-type protease in human vulva fibroblasts: its possible involvement with lichen sclerosus et atrophicus. J Invest Dermatol, 1982; 78: 270-5.
  300. Nelson DL, Balian G. The influence of retinoic acid on collagen synthesis by skin fibroblasts [Abstract]. J Invest Dermatol 1983; 80: 357.
  301. Davidson EA, Small W. Metabolism in vivo of connective-tissue mucopolysaccharides II. Biochum Biophys Acta 1963; 69: 453-8.
  302. Kaufman M, Straisfield C, Pinksy L. Expression of androgen-responsive properties in human skin fibroblast strains of genital and nongenital origin. Somat Cell Genet 1977; 3: 17-25.
  303. Friedrich EG, Kalra PS. Serum levels of sex hormones in vulvar lichen sclerosus and the effect of topical testosterone. N Engl J Med 1984; 308: 488-91.
  304. Strauss JS, Pochi PE. Recent advances in androgen metabolism and their relation to the skin. Arch Dermatol 1969; 100: 621-36.
  305. Meyer WJ, Migeon BR, Migeon CJ. Locus on human X chromosome for dihydrotestosterone recpetor and androgen sensitivity. Proc Natl Acad Sci USA 1975; 72: 1469-72.
  306. Sideri M, Parazzini F, Rognoni MT, et al. Risk factors for vulvar lichen sclerosus. Am J Obstet Gynecol 1989; 161: 38-42.
  307. Cummer CL. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1946; 54: 629.
  308. Hunt E. Diseases affecting the vulva. 4th ed. St Louis: CV Mosby, 1954: 92-9, 208.
  309. Falk AB, Bielinski S. Lichen sclerosus et atrophicus. Arch Dermatol 1964; 89: 619-20.
  310. Schoch EP, McQuiston CH. Diagnostic and therapeutic errors in certain dermatoses of the vulva. JAMA 1955; 157: 1102-6.
  311. Kaufman RH, Friedrich EG. The carbon dioxide laser in the treatment of vulvar disease. Clin Obstet Gynecol 1985; 28: 220-9.
  312. Ratz JL. Carbon dioxide laser treatment of balanitis xerotica obliterans. J Am Acad Dermatol 1984; 10: 925-8.
  313. Rosenberg SK, Jacobs H. Continuous wave carbon dioxide treatment of balanitis xerotica obliterans. Urology 1982; 19: 539-41.
  314. Mitchell JH. Lichen planus atrophicus. Arch Dermatol Syph 1929; 19: 143.
  315. Ginsburg L. Circumscribed scleroderma (white spot disease). Arch Dermatol Syph 1939: 39: 903-4.
  316. Feldman FF, Lerner AG. Bullous lichen sclerosus et atrophicus. Arch Dermatol 1961; 83: 705-6.
  317. Cummer CL. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1944; 49: 449.
  318. Turell R. Tattooing with mercuric sulfide for the treatment of intractable pruritis vulvae and ani. Am J Obstet Gynecol 1941; 42: 290-6.
  319. Dobes WL. Lichen sclerosus et atrophicus of the female genitalia [Comment]. Arch Dermatol 1962; 85: 372-3.
  320. Ward GD, Suthherst JR. Pruritis vulvae: treatment by multiple intradermal alcohol injections. Br J Dermatol 1975; 93: 201-4.
  321. Woodruff JD, Thompson B. Local alcohol injection in the treatment of vulvar pruritis. Obstet Gynecol 1972; 40: 18-22.
  322. Ridley CM. The vulva. London: WB Saunders, 1975: 172-85.
  323. Bergfield WF, Lesowitz SA. Lichen sclerosus et atrophicus. Arch Dermatol 1970; 101: 247-8.
  324. Scully JP. Lichen sclerosus et atrophicus. Arch Dermatol 1958; 77: 763.
  325. Everett MA, Coffey CM. Intradermal administration of chloroquine. Arch Dermatol 1961; 83: 131-3.
  326. Coskey RJ. Dermatologic therapy: December 1983 through November 1984. J Am Acad Dermatol 1985; 12: 1045-71.
  327. Bergman A, Karram M, Bhatia NN. Local steroid application for hyperplastic dystrophy of the vulva. J Reprod Med 1988; 33: 542-4.
  328. McKay M. Vulvodynia versus pruritis vulvae. Clin Obstet Gynecol 1985; 28: 123-33.
  329. Mering JH. A surgical approach to intractable pruritis vulvae. Am J Obstet Gynecol 1952; 64: 619-27.
  330. Mering JH. Some further observations on wide skin undercutting for intractable pruritis vuvlae. Am J Obstet Gynecol 1956; 71: 386-90.
  331. Crow KD, Ellis J, Wynne EJC. Lichen sclerosus et atrophicus of the penis. Br J Dermatol 1980; 103 (suppl 18): 17.
  332. Glickman FS, Silvers SH. Generalized bullous and hemorrhagic lichen sclerosus et atrophicus. Cutis 1972; 10: 501-3.
  333. Stritzler C. Lichen sclerosus et atrophicans. Arch Dermatol 1961; 83: 1028.
  334. Pillsbury DM, Shelley WB. Intradermal steroid therapy. Arch Dermatol 1963; 87: 338-9.
  335. Poynter JH, Levy J. Balanitis xerotica obliterans: effective treatment with topical and sublesional corticosteroids. Br J Urol 1967; 39: 420-5.
  336. Cole HN. Balanitis xerotica obliterans [Comment]. Arch Dermatol Syph 1941; 44: 560.
  337. Richardson AC, Williams GA. Topical androgenic hormones in vulvar kraurosis-leukoplakia syndrome. Am J Obstet Gyencol 1958; 76: 791-9.
  338. Zelle K. Treatment of vulvar dystrophies with topical testosterone proprionate. Am J Obstet Gynecol 1971; 109: 570-3.
  339. Cascano EF, Montes LF, Mazzini MA. Tissue changes in lichen sclerosus in children following local application of oestrogens. Br J Dermatol 1964; 76: 496-7.
  340. Friedrich EG. Topical testosterone for benign vulvar dystrophy. Obstet Gynecol 1971; 37: 677-86.
  341. Cinberg BL. Postmenopausal pruritis vulvae. Am J Obstet Gynecol 1945; 49: 647-57.
  342. Flynt J, Gallup DG. Childhood lichen sclerosus. Obstet Gynecol 1979; 53 (suppl): 79S-81S.
  343. Rompannen U. Long-term results of etretinate therapy in vulvar dystrophy. Curr Ther Res 1986; 40: 442-7.
  344. Jasionowski EA, Jasionowski P. Topical progesterone in treatment of vulvar dystrophy. Am J Obstet Gynecol 1977; 127: 667-70.
  345. Jasionowski EA, Jasionowski PA. Further observations on the effect of topical progesterone on vulvar disease. Am J Obstet Gynecol 1979; 134: 565-7.
  346. Brodey A. Hemorrhagic and bullous lichen sclerosus et atrophicus. Arch Dermatol 1967; 95: 328-9.
  347. Hesseltine HC. Vitamin therapies in vulvar dermatoses. Am J Obstet Gynecol 1941; 42: 702-13.
  348. Pascher F, Sawicky HH, Silverberg MG, et al. Tocopherols (Vit E) for discoid lupus erythematosus and other dermatoses. J Invest Dermatol 1951; 17: 261-6.
  349. Swift BH. Achlorhydria as an aetiological factor in pruritis vulvae associated with kraurosis or leucoplakia. J Obstet Gynecol Br Emp 1936; 43: 1053-77.
  350. Hyams MN. Leukoplakia of the vulva [Comment]. Am J Obstet Gynecol 1947; 54: 558.
  351. Post CF, Jewell EW. Lichen sclerosus et atrophicus. Arch Dermatol 1960; 82: 128.
  352. Hyams MN, Bloom OH. Leukoplakia vulvae. Am J Obstet Gynecol 1947; 53: 214-20.
  353. Klauder JV. Lichen sclerosus et atrophicus of the female genitalia [Comment]. Arch Dermatol 1962; 85: 373.
  354. Neuhofer J, Fritsch P. Treatment of localized scleroderma and lichen sclerosus with etretinate. Acta Derm Venereol (Stockh) 1984; 64: 171-4.
  355. Mørk N, Jensen P, Hoel PS. Vulval lichen sclerosus et atrophicus treated with estretinate (Tigason®). Acta Derm Venereol (Stockh) 1986; 66: 363-5.
  356. Minimäki X, Kallioinen M, Oikarinen A. Etretinate reduces connective tissue degeneration in lichen sclerosus et atrophicus. Acta Derm Venereol (Stockh) 1989; 69: 439-42.
  357. Rompannen U, Tuimala R, Ellmén J, et al. Treatment of dystrophic changes of the vulva with etretinate or placebo. Curr Ther Res 1987; 42; 211-8.
  358. Crocker. Lichen planus: its variations, relations, and imitations. Br J Dermatol 1900; 12: 421.
  359. Kyrle J. Histo-biologie der menschichen haut und ihrer erkrankungen. Wien: Verlag von Julius Springer, 1927: 140-1.
  360. Foerster HR. Balanitis xerotica obliterans [Comment]. Arch Dermatol Syph 1941; 44: 559-60.
  361. Brain RT, Haber H. Lichen sclerosus et atrophicus. Br J Dermatol 1950; 62: 170-1.
  362. Skeer J. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1941; 44: 530-1.
  363. Hubler WR. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1946; 54: 628-9.
  364. Rothran S, Farney Davies E. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1951; 64: 653-4.
  365. Ebert MH. Lichen sclerosus et atrophicus. Arch Dermatol Syph 1951; 64: 654-5.
  366. Lawrence WD. Lichen sclerosus et atrophicus of the vulva. Obstet Gynecol 1959; 14: 65-7.
  367. Abrahams I. Lichen sclerosus et atrophicus. Arch Dermatol 1973; 108: 433.
  368. Svendsen IB. Lichen sclerosus et atrophicus of the anogenital region in a girl aged 4 years. Acta Derm Venereol (Stockh) 1954; 34: 321-9.
  369. Oliver EA, Finnerud X. Lichen planus sclerosus et atrophicus. Arch Dermatol Syph 1929; 19: 829.
  370. Fleisher Tl, Zeligman I. Cutaneous findings in phenylketonuria. Arch Dermatol 1960; 81: 898-903.
  371. de Oliviera JM, Saleiro V. Involucrin expression in vulvar lesions. J Reprod Med 1986; 31: 828-30.
  372. Yoshima H, Shinkai H, Shigeharu S, et al. Acidic glycosaminoglycans in skin and urine of a patient with wide spreaded [sic] lichen sclerosus et atrophicus. Arch Dermatol Res 1978; 263: 59-66.
  373. Anderson CR. Bullous lichen sclerosus et atrophicus. Arch Dermatol Syph 1944; 49: 423-6.
  374. Tompkins KJ, James WD. Persistent bullae on the penis of an elderly man. Arch Dermatol 1987; 123: 1391-6.
  375. Buchanan JD, Catterall MD, Briggs PC. Posthitis xertoica obliterans. J R Nav Med Serv 1979; 65: 136-9.
  376. Di Paola GR. The problem of so-called cancerous lesions of the vulva: ten years of prospective experience. Eur J Gynaec Oncol 1980; 1: 20-8.
  377. Miller NF, Riley GM, Stanley M. Leukoplakia of the vulva. Am J Obstet Gynecol 1952; 64: 768-79.

(File revised 26 June 2005)