ABSTRACT

Mastocytosis is a rare clonal disorder characterized by excessive accumulation of pathological mast cells (MCs) in one or multiple distinct organs and tissues, such as the skin, gastrointestinal tract, and/or bone marrow (BM). The pathogenesis of all forms of mastocytosis results from both chronic and episodic MC mediator release and excessive MC accumulation in one or more tissues. The clinical manifestations associated with MC mediator release are identical to those of an allergic reaction and anaphylaxis. The most important clinical sign during physical examination in cutaneous mastocytosis (CM) is Darier's sign. Bullous eruptions with haemorrhage can occur in patients with diffuse cutaneous mastocytosis (DCM) and maculopapular cutaneous mastocytosis (MPCM). Practical interventions, such as the use of lukewarm water for bathing, air conditioning during hot weather, and avoidance of triggers for MC degranulation, are precautionary measures that help to avoid allergic reactions and anaphylaxis. Short-term use of topical corticosteroids may be useful for temporarily decreasing the number of skin MCs.