Korean J Dermatol.  2007 Oct;45(10):1083-1086.

A Case of Autosomal Recessive Type 2 Pseudoxanthoma Elasticum Presenting Generalized Cutaneous Laxity

• Hwang SW
• Cho KJ
• Oh DJ
• Lee D
• Kim JW
• Park SW

Abstract

Pseudoxanthoma elasticum is a rare, heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural skin. An 18-year-old man presented cutis laxa-like marked wrinkling on the axillae, trunk and thighs for about 10 years. There was no similar skin lesion on his family members and no other systemic involvement was found. Histopathological examination of the abdomen revealed fragmentation and calcification of elastic fibers in the middle and lower dermis. Von Kossa staining showed calcium deposition along the altered elastic fibers. Herein, we report a rare case of autosomal recessive pseudoxanthoma elasticum presenting generalized cutaneous laxity.

Keyword

Autosomal recessive; Pseudoxanthoma elasticum; Generalized laxity

MeSH Terms

Abdomen
Adolescent
Axilla
Calcium
Dermis
Elastic Tissue
Humans
Pseudoxanthoma Elasticum*
Skin
Thigh
Calcium