Abstract

Case presentation: A 20-month-old toddler hospitalized for congenital heart disease and CMV-induced anemia manifested a progressive retiform purpura and necrosis of the 4 extremities beginning 3 days after the onset of unconsciousness. Dermatologic evaluation revealed multiple well-demarcated violaceous plaques in an asymmetric distribution over the distal aspects of the extremities with scattered blisters evolving into ulcers. Clinical suspicion was for a vasculitis vs vasculopathy vs autoimmune condition. Biopsies from multiple sites all revealed a cell-poor subepidermal blister with extensive overlying epidermal necrosis and necrosis of the eccrine sweat glands and ducts. No inflammatory infiltrate was present, and a diagnosis of coma blister was made. Discussion: Coma-induced blister formation with eccrine sweat gland necrosis is a rare condition that is predominantly associated with prolonged impairment of conscious level in adults. First described in association with barbiturate intoxication, it is now reported in as many as 4% to 5% of adult patients hospitalized for drug-induced coma . However, the condition is rare in children, with only 5 cases reported during the period from 1986 to 2012. The etiology of the condition is complex and cannot simply be explained by pressure effects. In children, only half of cases have been reported in pressure-prone areas, suggesting that other factors may be at play, including immune-mediated mechanisms, drug toxicity and metabolic imbalances. Typical histological features are necrosis of the epidermis, subepidermal blisters and eccrine sweat gland necrosis. Because of the rarity of the disease in children, cases are likely to go unrecognized. Coma blisters are self-limiting and benign, but it is important to correctly identify the lesions in children to rule-out other blistering diseases and avoid unnecessary treatment.

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