Hereditary progressive mucinous histiocytosis: Three different phenotypes in three family members

Abstract

We describe three cases of multiple histiocytic cutaneous tumors that began in childhood and affected three members from two generations of the same family: a mother, a daughter, and a nephew. The lesions were mostly skin-colored papules distributed symmetrically on the dorsum of the forearms and hands and on the face and thighs. There were no signs of spontaneous regression. The clinical and histopathological features were consistent with a diagnosis of hereditary progressive mucinous histiocytosis (HPMH), but phenotypic expression varied somewhat between the three patients. HPMH has only been described in eight families to date, and just one of the reports included three well-documented cases. Our cases confirm that HPMH can affect males and expands the clinical spectrum of skin lesions in this disease.

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