Pseudoxanthoma Elasticum

Author: Chen Tien-Hsing   Wei Fu-Chan  

Publisher: Informa Healthcare

ISSN: 0284-4311

Source: Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery, Vol.32, Iss.4, 1998-11, pp. : 421-424

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Abstract

Pseudoxanthoma elasticum is an uncommon disorder of elastin with characteristic skin laxity. Rhytidectomy of the excessive redundant skin is necessary for cosmetic improvement, and reports of surgical management are rare. We report a case of pseudoxanthoma elasticum with involvement of neck, axillae, breasts, abdomen, thighs, and perineum, together with angioid streaking of fundi. Rhytidectomy of the lax cervical skin was undertaken with satisfactory results after a 13 month follow-up. We have reviewed other published reports to obtain a better understanding of the genetics, histopathology, vascular and ophthalmological manifestations, and also the surgical implications of pseudoxanthoma elasticum.