" />

Paediatric cutaneous mastocytosis-A rare case report and approach to diagnosis


Article PDF :

Veiw Full Text PDF

Article type :

Case Report

Author :

Swati Raj, Naveen Thapliyal

Volume :

10

Issue :

1

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.

Keyword :

">