Mastocytosis is a diverse group of rare disorders characterized by mast cell proliferation and its aberrant accumulation within various organs including respiratory, gastrointestinal, genitourinary mucosa and most commonly the skin. The spectrum of presentations ranges from torpid disease (cutaneous mastocytosis) having normal life span with transient sign and symptoms to highly vigorous disease (systemic mastocytosis) and life-threatening malignant conditions. Prevalence of the disease in general population is unknown. It occurs in all races and there is no sex predilection. Peak incidence is during infancy and early childhood with second peak occurring in middle age. We report a case of maculopapular cutaneous mastocytosis (MCM) in a six-month-old male child who presented with respiratory distress. According to our literature search it is one of the least frequent cases reported in our country, Pakistan.
Keywords: maculopapular cutaneous mastocytosis; pakistan; respiratory distress; skin.