Abstract

Mastocytosis is a disorder characterized by abnormal mast cell proliferation. The skin is the most common site of involvement. Extracutaneous involvement occurs in systemic mastocytosis with infiltrations in the gastrointestinal tract, lymph nodes, bone marrow, liver, and spleen which result in systemic manifestations and life-threatening complications. The most common form of skin lesions in mastocytosis is urticaria pigmentosa (UP). Other less common forms include diffuse cutaneous mastocytosis (DCM), mastocytoma and telangiectasia macularis eruptive perstans (TMEP). Bullae can develop in skin lesions of mastocytosis. When bullae are the main presenting feature, the term bullous mastocytosis is used. Bullous mastocytosis can cause diagnostic confusion as it can be mistaken with other bullous skin diseases. Herein we present a case of bullous mastocytosis that was initially thought to be a form of child abuse. We emphasize the importance of skin biopsy in the diagnosis of this form of mastocytosis..

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