Indolent systemic mastocytosis.

نویسندگان

Sergio Vano-Galvan

Belén De la Hoz

Rosa Nuñez

Pedro Jaen

چکیده

circular macules, less than 1 cm in diameter, located on his trunk and extremities [Figure A]. Wheals and surrounding erythema developed in the lesions after rubbing (positive Darier sign). Laboratory analyses showed mild eosinophilia with an increased total tryptase level of 40 ng/ml. Densitometry was normal. With the strong clinical suspicion of cutaneous mastocytosis, a skin biopsy was performed. Histological examination [Figure B] using immum astocytosis is a rare disease. Because of this, general practitioners have limited exposure to its clinical manifestations and management. Mastocytosis is characterized by an accumulation of mast cells in the skin and/or other tissues. The most frequent form of mastocytosis is cutaneous, followed by indolent systemic mastocytosis. Depending on the extent of the disease, it may present with symptoms resulting from mast cell degranulation, including flushing, diarrhea, vomiting, syncope, or anaphylaxis. We present the case of a young man with this rare disease, and review the literature on the more interesting aspects of the disorder for general practitioners.

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