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Mastocytosis : Introduction

 
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CutaneousMastocytosis22.jpg

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Introduction: Mastocytosis is a clonal proliferation and accumulation of sheets or compact clusters of neoplastic mast cells in one or more organs. There are two main forms of the disease and many subtypes - cutaneous mastocytosis (80% of cases) in which the mast cell infiltrates are limited to the skin, and systemic mastocytosis (20% of cases) with involvement of at least one extracutaneous organ (usually bone marrow), although skin lesions are present in about 50% of cases.

Mastocytosis is a heterogenous disease. One end of the spectrum consists of asymptomatic or spontaneously regressing benign and solitary lesions with normal life expectancy, while the other end comprises of highly aggressive forms with multiorgan failure and poor survival.

The image shows a dense dermal infiltrate of mast cells in urticaria pigmentosa - the commonest form of mastocytosis and accounting for about 80% of cutaneous cases.

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