Bullous mastocytosis: A fatal outcome

A 6-week-old boy was referred with a generalized bullous rash since birth. Examination revealed bullous mastocytosis with initially no evidence of sys temic involvement. Hepatosplenomegaly was noted at 6 months, and at 12 mont hs he was found to have generalized lymphadenopathy. He developed bouts of vomiting associated with increased blistering. At 17 months he had sudden c ollapse following a brief bout of vomiting and was apneic and asystolic on arrival at the emergency department. The cause of death was attributed to m assive hypotension secondary to mast cell degranulation, Although childhood mastocytosis has a favorable course in general, the subset of children wit h congenital bullous mastocytosis is at higher risk of sudden death and a m ore guarded prognosis should be given.