Diffuse cutaneous mastocytosis: pseudoxanthomatous variant

Diffuse cutaneous mastocytosis is one of the extremely rare benign forms of mastocytosis that have varied clinical presentations. The pseudoxanthomatous variant is an extremely rare multinodular nonpigmented entity. Only two cases have been reported so far in the literature. We report a 21 year old female patient who had diffusely infiltrated skin with multiple papulo-nodular lesions resembling xanthomas and the classical histopathological features of mastocytosis.