IngentaConnect A sporadic case of progressive mucinous histiocytosis

Authors: Sass, U.¹; André, J.¹; Song, M.¹

Source: British Journal of Dermatology, Volume 142, Number 1, January 2000 , pp. 133-137(5)

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.

Keywords: differential diagnosis; hereditary progressive mucinous histiocytosis

Document Type: Research article

DOI: 10.1046/j.1365-2133.2000.03255.x

Affiliations: 1: Department of Dermatology, C.H.U. Saint-Pierre, Free University of Brussels, Boulevard de Waterloo 129, B-1000 Brussels, Belgium

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