Saba Khanum, Juwairiya Ilyas, Faseeha Eram
Pediatric MPCM is rare, often under-recognized, and may mimic other dermatoses. This case highlights the importance of a structured diagnostic approach, exclusion of systemic disease, and comprehensive parental counseling. Early recognition and tailored management are critical to improving outcomes and reducing unnecessary anxiety.
INTRODUCTION: Mastocytosis is a rare disorder characterized by abnormal mast cell proliferation, with clinical manifestations ranging from isolated cutaneous disease to systemic involvement. In children, it most often presents as cutaneous mastocytosis (CM), which is a usually benign and self-limiting condition. However, accurate recognition is essential to exclude systemic mastocytosis.
CASE PRESENTATION: We report a 9-month-old male infant with multiple hyperpigmented macules on the trunk and extremities, accompanied by a positive Darier's sign. A diagnostic work-up, including clinical examination, serum tryptase measurement, histopathology, and immunohistochemistry, confirmed maculopapular cutaneous mastocytosis (MPCM, aka urticaria pigmentosa).
RESULTS: The child had no systemic symptoms, and serum tryptase was mildly elevated, supporting a favorable prognosis.
MANAGEMENT AND OUTCOME: Management was conservative and focused on symptomatic relief with antihistamines, parental education, and trigger avoidance. Unani preparations (Sharbat Unnab and Roghan Gul) were prescribed as adjuncts for symptomatic relief. Parents were reassured of the benign, self-limiting nature of pediatric CM. However, the patient was lost to follow-up.
CONCLUSION: Pediatric MPCM is rare, often under-recognized, and may mimic other dermatoses. This case highlights the importance of a structured diagnostic approach, exclusion of systemic disease, and comprehensive parental counseling. Early recognition and tailored management are critical to improving outcomes and reducing unnecessary anxiety.
KEYWORDS: Pediatric dermatology, Cutaneous Mastocytosis, Urticaria pigmentosa, Darier's sign, Serum tryptase, Histopathology, Unani medicine, Case report.