Mastocytosis represents a heterogeneous group of clinical disorders resulti
ng from the infiltration of mast cells in the skin and other organs. Althou
gh mastocytosis was first described over 130 years ago, the pathophysiologi
c mechanisms responsible for this disease have been identified only recentl
y. This article discusses the salient clinical features of the disease, the
mechanisms responsible for its development, and provides treatment approac
hes that have proven useful for managing patients with this disorder.