Coexistence of pseudoxanthoma elasticum-like papillary dermal elastolysis and linear focal dermal elastosis

An 89-year-old Japanese woman bad pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck and linear focal elastosis on the flavor aspects of the legs and thighs, The lesions of both diseases had similar histologic patterns-loss of elastic fibers in the papillary dermis and accumulation of normal-appearing elastic fibers in the subpapillary or mid dermis. Immunohistochemical analysis showed elastin, and microfibril-associated proteins (fibrillin 1 and 2 and microfibril associated glycoprotein 1 and 4) were absent or decreased in the papillary dermis and present in the accumulated elastic fibers in both lesions. The association of 2 diseases on different areas of the same patient and histologic and immunohistologic similarities between the diseases suggest that, despite the clinical differences, both diseases are closely related.