Abstract :
Mastocytoses are described as a heterogeneous group of disorders characterized by clonal proliferation and accumulation of phenotypically atypical mast cells in one or more organs.Mastocytoses are categorized into two broad categories: cutaneous mastocytosis (CM) and systemic mastocytosis (SM).Paediatric mastocytosis usually presents before 2 years of age in 90% and in a few at birth.Approximately 15% of cases are congenital. Mastocytoses affect all races with gender predilection; however, few of the studies indicated a slight male predominance.CM is most common in the paediatric age group, representing 90% of the cases involving only skin in the absence of systemic presentation. SM is associated with haematological malignancy, commonly seen in adult-onset mastocytosis.In 2019, WHO issued the latest classification of mastocytosis. Literature testifies to the rarity of data of CM from India. We present a case report in a 3-year-old child from the Garhwal region of North India. The aim of our case report is to illuminate the diagnostic approach in paediatric mastocytosis as per current WHO guidelines.
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