Track: Case Reports

Abstract:

A 71-year-old male with a history of alcoholic cirrhosis sustained a mechanical fall and developed a periprosthetic knee infection, which grew Corynebacterium striatum. He was started on broad-spectrum antibiotics, which were eventually narrowed to a six-week course of vancomycin. Sixteen days later, the patient presented with erythematous and dusky papules coalescing into full-thickness desquamation affecting the trunk and extremities. A punch biopsy revealed an orthokeratotic stratum corneum with dyskeratotic keratinocytes at multiple levels of the epidermis and a sparse superficial perivascular, predominantly lymphocytic infiltrate. Given the clinical presentation and consistent pathology, a diagnosis of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) was rendered. However, later a direct immunofluorescence (DIF) of perilesional skin resulted and demonstrated strong linear deposition of IgA along the basement membrane, supporting a diagnosis of SJS/TEN-like linear IgA bullous dermatosis (LABD). The SJS/TEN-like presentation of LABD is exceptionally rare and has only approximately 20 reported cases. This is a difficult diagnosis to render as the clinical and histologic presentation of SJS/TEN and SJS/TEN-like LABD are indistinguishable without DIF. Cases of SJS/TEN-like LABD are noted to have a positive Nikolsky sign, mucosal involvement, and an onset range of several weeks. Further, on histologic inspection, a neutrophilic infiltrate may be sparse or absent. An accurate diagnosis in these cases can be critical in terms of recognizing the correct medication for withdrawal as well as further medical management. We present this case to highlight a rare presentation of LABD to improve clinician recognition of this uniquely challenging entity.

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Brian Cheng