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Development and calcification of skin lesions in 39 patients with pseudoxanthoma elasticum


, : Development and calcification of skin lesions in 39 patients with pseudoxanthoma elasticum. Clinical & Experimental Dermatology 4(3): 291-302

Twelve male and 27 female patients 9-56 yr old with pseudoxanthoma elasticum (PXE) were studied. In 1/2 of the patients, PXE presented before age 10. Discrete lesions first appeared in sites of predilection (neck, axilla, antecubital fossa, groin and popliteal fossa) and then spread marginally at varying rates. Affected skin area paralleled duration of disease rather than age. Dermal Ca in PXE-affected neck skin ranged from 3 to several hundred times normal levels but was significantly raised in clinically unaffected back skin of 1/3 of the patients. The rate of spread of skin lesions was unrelated to Ca level or to Ca uptake in vitro of affected neck dermis. Skin PXE depends on initiation of discrete lesions followed by a 2nd processs leading to their marginal spread.

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