Urticaria pigmentosa.

Urticaria pigmentosa (UP), resulting from the accumulation of excessive numbers of mast cells in the skin, is the most common form of cutaneous mastocytosis. Observations highlight the diversity of this disease. Clonal expansion of early hematopoietic progenitor cells carrying activating mutations in KIT seems to be the basis of adult-onset UP. New pathogenetic findings are leading to the development of new diagnostic surrogate markers of disease and therapeutic approaches targeting neoplastic mast cells. Promising strategies may arise from an increased understanding about the cause of mastocytosis and the signaling pathways initiated by kit activation.