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.
ALTMAN J, WINKELMANN RK. Xanthomatous Cutaneous Lesions of Histiocytosis X. Arch Dermatol. 1963;87(2):164–170. doi:10.1001/archderm.1963.01590140026004
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