ABSTRACT

We report on a 9-month-old male infant with multiple tense bullae, erosions and hemorrhagic crusts on the scalp, face and distal lower limb. He had generalised yellowish thick and rough skin, giving doughy feel and 'peau d' orange' appearance of the skin at places. The histopathological examination revealed a dense dermal cellular infiltrate identified as mast cells by toluidine blue stain. The direct immunofluorescence was negative. A diagnosis of diffuse cutaneous mastocytosis (DCM) with generalized bullae was made based on these clinical and histopathological findings. In cases with DCM with generalized bullae, systemic involvement is more frequent and more severe compared to other types of cutaneous mastocytosis. Some lethal outcomes have been reported. Therefore, proper follow-up with evaluations for systemic involvement is required for these patients.