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February 1963

Xanthomatous Cutaneous Lesions of Histiocytosis X

Arch Dermatol. 1963;87(2):164-170. doi:10.1001/archderm.1963.01590140026004

The cutaneous manifestations of histiocytosis X, particularly the Hand-Schüller-Christian type, are reviewed. Xanthomatous-appearing lesions, which are rare with histiocytosis X, can be classified according to their clinical and histologic features as (I) yellowish papular and seborrheic lesions; (2) xanthoma disseminatum; (3) juvenile xanthogranuloma; (4) plane, papular, and tuberous xanthomata secondary to complicating biliary cirrhosis; and (5) xanthelasma palpebrarum. The multiple histopathologic patterns that may be found in biopsies of such lesions are illustrated.

The rare cases of histiocytosis X associated with cutaneous lesions that show features consistent with xanthoma disseminatum, juvenile xanthogranuloma, and reticulohistiocytoma suggest that these various entities are related clinicopathologic reaction patterns which form a spectrum of histiocytic proliferative disease. Although in most instances these reaction patterns are quite distinct, the occasional occurrence of cases with overlapping features should not be surprising.