Mastocytosis is a group of disorders characterized by an abnormal accumulation of mast cells in one or more organ systems. Cutaneous mastocytosis, by definition, is the restriction of mast cells to the skin although patients with this disease may have both cutaneous and systemic symptoms. Recognition of cutaneous mastocytosis is important, especially in children, as its prognosis is favorable in the majority of cases, and, while there is no cure, treatment options are available for symptomatic relief. Annual visits to monitor for signs of progression to systemic disease as well as patient education about potential triggers and consequences of mast cell degranulation are an important component of management.
KeywordsCutaneous mastocytosis Urticaria pigmentosa Mastocytoma Tryptase Telangiectasia macularis eruptiva perstans Darier’s sign
We gratefully thank Kord Honda, MD, Assistant Professor of Dermatology and Director of Dermatopathology at Case Western Reserve University, for providing the images of mastocytosis histology.
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