title: A sporadic case of progressive mucinous histiocytosis.
abstract: Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. Light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. Iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. Immunohistochemistry showed positive labelling with alpha-1 antitrypsin, Factor XIIIa and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.
authors.affiliation: Department of Dermatology, C.H.U. Saint-Pierre, Free University of Brussels, Boulevard de Waterloo 129, B-1000 Brussels, Belgium.
authors.forename: U
authors.initials: U
authors.lastname: Sass
authors.forename: J
authors.initials: J
authors.lastname: André
authors.forename: M
authors.initials: M
authors.lastname: Song
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D015614:Histiocytosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D009077:Mucins / Q000378:metabolism; D012871:Skin Diseases / Q000473:pathology*
chemical_list: D009077:Mucins