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Case Reports

Abstract

Our case is of a 39-year-old man with developmental delay who presented with an abrupt-onset erythroderma, superficial erosions on the trunk and thighs, and glans penis ulceration. Skin biopsies revealed full-thickness epidermal necrosis, dyskeratotic keratinocytes with overlying orthokeratosis, and superficial perivascular inflammation comprised of lymphocytes, plasma cells, and scattered eosinophils. These findings are most suggestive of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). SJS/TEN is a severe mucocutaneous reaction, usually drug-induced, characterized by extensive epidermal and mucosal necrosis and detachment. Histologically, it features full-thickness epidermal necrosis, dyskeratotic keratinocytes, and subepidermal blistering. Although histologic findings of our case were consistent with SJS/TEN, our patient had no known inciting medication and a history of zinc deficiency with low-normal serum zinc. Empiric zinc supplementation was initiated, leading to clinical improvement. Zinc deficiency can induce keratinocyte apoptosis through caspase-dependent pathways and can lead to acquired acrodermatitis enteropathica (AAE). AAE is a nutritional dermatosis of zinc deficiency with characteristic histology of pallor, vacuolization, and ballooning degeneration of the upper epidermis. AAE typically presents with scaly, erythematous, or vesicobullous plaques in intertriginous, periorificial, and acral sites. To our knowledge, this is the first reported case of a nutritional deficiency mimicking the clinical and histological presentation of SJS/TEN. Given the absence of a drug trigger, the presence of zinc deficiency, and the patient’s response to zinc supplementation, we propose this case may represent a robust, atypical manifestation of AAE with SJS/TEN-like features. This case highlights the importance of considering nutritional deficiencies in the differential diagnosis of severe mucocutaneous reactions.