ABSTRACT

Mastocytosis is characterized by abnormal growth and accumulation of mast cells in one or multiple organs. In children, mastocytosis is commonly cutaneous and the most frequent clinical presentation form is urticaria pigmentosa. Urticaria pigmentosa is a generally benign disease. It often occurs in the first 2 years of life and in most patients remission is observed in puberty. Extracutaneous organ involvement and progression to systemic mastocytosis is not common in children. Mast cell mediator-related symptoms such as pruritus, urticaria and flushing may occur in patients. Flares of lesions are induced by sudden temperature changes, physical stimuli, anxiety, exercise and medications. Symptoms respond to topical and systemic anti-mediator therapy including histamine receptor antagonists and cromolyn sodium. The diagnosis is based on clinical and histopathological findings. Herein, since it is an uncommon disease we report an 11-month-old girl with urticaria pigmentosa having its typical clinical and histological features.