Staphylococcal scalded skin syndrome

A complication of circumcision.

David Annunziato, MD, Louis M. Goldblum, DO.

• Numerous complications have been reported as a result of neonatal circumcision. We describe here three cases of staphylococcal scalded skin syndrome that were thought to be due to infected circumcisions. A review of the literature failed to disclose descriptions of staphylococcal scalded skin syndrome as such a complication. In spite of recent American Academy of Pediatrics recommendations against routine circumcision, it will continue to be a common procedure. Careful preoperative and postoperative screening and guidance are urged to prevent staphylococcal scalded skin syndrome and other complications.

Neonatal circumcision is the most common surgical procedure in the United States today. Many complications have been reported to be associated with this procedure. Recently, we encountered two cases of staphylococcal scalded skin syndrome as complications of circumcision in otherwise healthy full-term infants. Each case had the essential clinical features of staphylococcal scalded skin syndrome. A third case, though unproved, was believed to be clinically the same.

An extensive review of the literature failed to reveal reports of staphylococcal scalded skin syndrome as a complication following circumcision. We hope to alert physicians caring for newborns to this possibility.

Over the years, when advising parents of the problems encountered after circumcision, we had not considered staphylococcal scalded skin syndrome. Indeed, we have even reassured parents as to the paucity of problems after “ritual” circumcisions.

REPORT OF CASES

    Case 1.–A 13-day-old male infant was admitted because of “high fever to 40.6°C” and generalized “peeling of the skin.” Discharged on the fourth day of life, he developed two pustules in his groin and on the shaft of the penis six days prior to admission, but he was “doing fine” and thriving otherwise. Four day prior to admission he underwent a ritual circumcision performed by a Mohel. The following day, more pustules developed on the lower abdomen and about the circumcision site. The mother also noted that the skin was “red” and easy to “peel off.” Their pediatrician recommended immediate hospitalization.
     On admission, the infant was hypothermic and in profound shock. The skin was erythematous, almost violaceous in areas, desquamating and tender. Nikolsky’s sign was present. The circumcised area was red, edematous, and exuding yellow pus (Fig 1). Cultures were taken and parenteral administration of methacillin nad gentamicin begun. He was treated aggressively with electrolyte solutions, whole blood, other plasma expanders, vasopressors, and mechanical ventilatory support. In spite of this he died 41 hours after admission. Staphylococcus aureus was cultured from the nasopharynx, throat, pustules, circumcised area, and conjunctivae. Blood cultures were negative.
     Case 2.–A full-term 3-day-old male infant was transferred to our medical center with a history of diarrhea and peeling of his skin beginning that day. On the second day of life, he was circumcised with a commercial clamp; on the third day (28 hours after circumcision), he had two explosive watery stools, and the skin of the penis, scrotum, buttocks, and periumbilical area were noted to be red and desquamating (Fig 2). The circumcised area was red and covered with a profuse, thick, yellow-green exudate. Nikolsky’s sign became positive over his entire body surface. Parenteral administration of methicillin was begun, along with fluids and electrolyte replacement. He responded well and was discharged after 13 days. Staphylococcus aureus grew from the cultures of the infants nasopharynx, the circumcised area, and from the mother’s nose.
     Case 3.–A 4,320-g, 2-day-old male infant was transferred to our medical center for evaluation of possible sepsis. At 31 hours of age, he was circumcised with a commercial clamp. Several hours later, he was noted to feed poorly and became lethargic, febrile to 39.4°C, and cyanotic in room air. A generalized erythematous maculoapapular rash erupted. The circumcised area was obviously infected, appearing red, swollen, hot and covered with a heavy, yellow, purulent exudate. No other site of infection was found. The usual and well as infected sites were cultured and antibiotic therapy begun. The rash became confluent. The skin was edematous and very tender. Within two days, the rash began to fade, and by the fourth hospital day, it cleared. While the skin never desquamated, it appeared like that of an early staphylococcal scalded skin syndrome (Fig 3). The cultures from the nasopharynx and circumcision site from S aureus. The infant responded well to therapy.

[CIRP Note: The three figures are not available.]

COMMENT

The etiology and pathophysiology of staphylococcal scalded skin syndrome were a mystery to Gottfried Ritter von Rittershain¹ when he originally described 297 cases of this entitity, which became known as Ritter’s disease.¹ Recently, 100 years later, it has been recognized that most cases are due to infection with S aureus, known to release an exotoxin, exfoliation.² This toxin causes a separation of the epidermis from the basement membrane at a superficial level and leads to the classic Nikolsky’s sign. Other names ascribed to the condition are toxic epidermal necrolysis and Lyell’s disease, the latter usually being applied to the condition in adults.

We thought that case 3 was similar to the 11 described by Melish and Glasgow² as a milder variant of the exfoliative form of staphylococcus scalded skin syndrome.

According to the 1971 report of the Ad Hoc Task Force on Circumcision of the American Academy of Pediatrics, “Circumcision is a surgical procedure that requires careful aseptic technique, systematized post-operative observation, and evaluation after discharge from the hospital. The immediate hazards of the newborn include local infection which may progress to septicemia, significant hemorrhage, and mutilation.”³

Many complications of elective circumcision are reported. Hemorrhage is the common complication; local infection ranks second in frequency.⁴ This latter complication is manifested by local inflammatory changes, ulceration, and suppuration. More severe infections have been reported, including partial necrosis of the penis, staphylococcal septicemia with osteomylitis, and pulmonary abscesses.⁵ The open wounds of circumcision have been reported as a source of neonatal sepsis in prematures.⁶

Other, although rare, complications reported include urethral fistula,⁷ circumcision of unrecognized hypospadias,⁴ laceration of the penile and scrotal skin,⁵ wound dehiscence,⁴ urinary retention from a too-tight dressing,⁵ injury to the glans,⁶ retention of plastic device ring with edema of the penile shaft,⁸ and surgical bivalving of the either the dorsal or ventral half of the glans while making the “dorsal slit.”⁹

Staphylococcal scalded skin syndrome is caused by a staphylococcal exotoxin that is elaborated at the site of infection. In the three cases presented, the circumcision site was the probable focus. As reported, neonates and infants are the predominant victims for almost all cases. The patients have not exhibited any antecedent or subsequent immunological or metabolic deficiencies.¹⁰

Since this disease can be rapidly fatal if untreated, as illustrated by case 1, accurate diagnosis and treatment are necessary. It is urged that careful scrutiny of the neonate’s total skin skin surface be made prior to circumcision. This was clearly omitted in case 1. Any rash, especially pustulosis, should force the operator to delay the procedure, as should be the presence of staphylococcal infection in the nursery.

In spite of recent recommendations against circumcision³ except for specific medical and religious requirements, it has and will continue to be a common surgical procedure. Awareness of its potential hazards, scrupulous preoperative examination, detailed instructions to the parents regarding postoperative care, and observations for signs of complications are vital to an uneventful outcome.

References

       1. Ritter von Rittershain G: Die exfoliative Dermatitis Jungerer Sauglinge. Centralzeitung Kinderheilkd 2:3-23, 1878.
       2. Melish ME, Glasgow LA: Staphylococcal scalded syndrome: Development of an experimental model. N Engl J Med 282:1114-1119, 1970.
       3. Report of the Ad Hoc Task Force on Circumcision. Pediatrics 56:610-611, 1975.
       4. Gee WF, Ansell JS: Neonatal circumcision: A ten-year overview: With comparison of the Gomco clamp and Plastibell device. Pediatrics 58:824-827, 1976.
       5. Shulman J, Ben Hun R, Neuman Z: Surgical complications of circumcision: Am J Dis Child 107; 149-154, 1964.
       6. Kirkpatrick BV, Eitzman DV: Neonatal septicemia after circumcision. Clin Pediatr 13:767-768, 1974.
       7. Lackey JT, Manniou RA: Urethral fistula following circumcision. JAMA 206:2318, 1968.
       8. Rubenstein M, Bason WM. Complications of circumcision done with a plastic bell clamp. Am J Dis Child 116:381-383, 1968.
       9. McGowan AJ Jr: A complication of circumcision. JAMA 207:2104-210, 1969.
     10. Elias PJ, Fritsch P, Epstein EH Jr: Staphylococcal scalded skin syndrome. Arch Dermatol 113:207-219, 1977. [Medline]

---

    From the Department of Pediatrics, Nassau County Medical Center, East Meadow, NY and the Department of Pediatrics, State University of New York, Stony Point Health Sciences Center (Dr Annunziato).
     Reprint requests to Department of Pediatrics, Nassau County Medical Center, 2201 Hempstead Turnpike, East Meadow, NY 11554 (Dr Annunziato).