Toxic epidermal necrolysis in an 8-year-old girl successfully treated with cyclosporin A, intravenous immunoglobulin and plasma exchange

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two rare, acute and severe dermatoses. They are characterized by different extent of epidermal necrolysis. Toxic epidermal necrolysis is associated with high mortality and it is one of the most severe disorders in dermatology. The most frequent and predominant triggers of TEN are drugs. The annual risk of TEN in the general population is calculated as 0.4–1.2 per million [1, 2]. The prevalence of this toxic reaction is unknown in children. It looks likely to be less frequent than in the adult population [1, 2]. The treatment of TEN is difficult and there is no optimal management established. Several therapy modalities have been proposed and analyzed [2]. We present a case of very severe TEN in an 8-year-old child successfully treated with combination therapy of cyclosporin A, intravenous immunoglobulin and plasma exchange. We would like also to draw attention to the difficulties in diagnosing TEN in such young patients.
An 8-year-old girl in a very serious condition was admitted to the Pediatric Intensive Care Unit (PICU).
Before admission she had been hospitalized in the Department of Infectious Diseases. On the basis of clinical manifestations (high fever, cough, photophobia, blistering skin eruptions on erythematous background) and laboratory tests (C-reactive protein 209 mg/dl, norm: > 5 mg/dl; procalcitonin 11.03, norm: 0.05, aspartate aminotransferase 106 IU/l, norm: 0–45 IU/l, alanine aminotransferase 141 IU/l, norm: 0–40) staphylococcal scalded skin syndrome was diagnosed and therapy with amoxicillin 90 mg/kg/day was initiated.
On admission to the PICU the girl was conscious with efficiency of circulatory and respiratory systems. Erythematous and blistering eruptions on the face, trunk and extremities (encompassing about 90% of total body surface area) were observed (Figure 1). Nikolsky’s sign was highly positive. Bloody erosions on all mucous membranes (in the oral cavity, genital region and eyes) were also visible (Figure 2).
During the first day of hospitalization in the PICU the girl was still treated as having staphylococcal infection. The cultures of the skin, throat and blood were taken. Antibiotic therapy was continued. However her clinical condition worsened. Cultures were negative. The biopsy from the skin lesion was taken. Because of the respiratory and circulatory insufficiency, the girl was intubated and pharmacological treatment with...


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