Surgical resection of a solitary cutaneous mastocytoma with resolution of apnea and multi-system symptoms

Abstract

Solitary cutaneous mastocytoma (SCM), one of the varieties of mastocytosis, presents as an about 2–4 cm long, reddish brown skin lesion with peau d'orange appearance. The lesion may erupt into blisters, urticaria or generalized cutaneous erythematous rash spontaneously, or upon stroking. The symptomatology is consequent to acute mast cell degranulation and histamine release. The standard treatment of SCM is non-surgical and includes H2 blocking agents and topical steroids. We report a case of SCM in a 2 months old child, which presented with recurrent apnea and additional heterogeneous symptoms involving respiratory, gastrointestinal and neurological systems. The symptoms resolved completely with surgical excision of the lesion at age six months. Such atypical multi systemic clinical presentation with acute life threatening symptoms, as well as surgical excision as a successful therapeutic intervention were not reported before in SCM.