Abstract
Progressive mucinous histiocytosis (PMH) is a very rare, benign form of non‐Langerhans' cell histiocytosis of the skin. Patients show characteristic 2‐10 mm sized red‐brown and flesh colored, non‐pruritic papules, particularly on the back of the hands and the extensor sides of the forearms. Up to date seventeen respectively fifteen hereditary cases in seven respectively eight2 families and five sporadic cases have been reported. Pathogenetically, a lysosomal storage disease of histiocytes, as well as proliferation and accumulation of histiocytes to a yet undetected stimulus are considered.
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