| Summary: | Introduction. Mastocytosis is a group of rare diseases characterized byabnormal growth of mast cells (MC) in the skin, bone marrow, liver,spleen and lymph nodes. In children cutaneous mastocytosis (CM) isthe predominant form.Objective. Presentation of four atypical cases of childhood CM with variousclinical manifestations to demonstrate the wide spectrum of skinlesions and MC mediator-related symptoms and discuss indications toperform diagnostic procedures for systemic mastocytosis (SM) in children.Case reports. We describe the clinical presentation and diagnostic proceduresperformed in 3 girls and 1 boy aged from 9 month to 6 years.Disseminated CM with bullous lesions, large mastocytoma, plaquetype and nodular type of CM were diagnosed. Elevated serum tryptase level and severe MC mediator-related symptoms were present only ina girl with extensive skin involvement and periodic blistering. SM oranaphylactic shock in the case history were not present in our children.Conclusions. Atypical clinical manifestations in infants and small childrenmake the diagnosis of CM difficult. Initial diagnosis of SM,follow-up and prevention of anaphylaxis are recommended in paediatricmastocytosis cases.
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