Challenges in Cutaneous Mastocytosis

Cutaneous lesions of mastocytosis (CLM) are a hallmark for the diagnosis of mastocytosis. The nomenclature of the term "cutaneous mastocytosis" (CM) is ambiguous as it is used both for classification excluding and as a morphologic description including systemic variants of mastocytosis. Recognition of CLM may be delayed by clinically subtle and very heterogeneous presentations. Distinguishing monomorphic versus polymorphic maculopapular CM may be challenging. Less common presentations require a clearly positive Darier's sign, histopathological confirmation, and/or skin D816V kit mutational analysis. Management of patients with CLM requires multidisciplinary therapy, adequate staging, and follow-up to recognize complications.