Abstract

Mastocytosis belongs to a group of rare diseases in which there is a pathological accumulation of mast cells in tissues. In childhood, it is more often limited to skin lesions. A clinical case of a bullous form of severe cutaneous mastocytosis, with the onset of the disease at 3.5 months, is presented. Cutaneous manifestations with a positive Darier–Unna symptom and a high level of tryptase in the blood, the exclusion of systemic lesions allowed diagnosing cutaneous mastocytosis and choosing treatment tactics.

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