Epidermal nevus syndrome is a group of congenital syndromes comprising epidermal nevus in conjunction with central nervous system, ocular, musculoskeletal, cardiac and renal abnormalities. Mastocytosis is a disease characterized by an abnormal increase of mast cells in one or more organ systems. The skin is the most frequently involved organ in patients with mastocytosis. We report here a rare case of epidermal nevus syndrome with cutaneous mastocytosis.
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