Osteoma cutis coexisting with cutis laxa-like pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic fibers ch aracterized by yellowish, coalescing papules on the loose and wrinkled flex ural area. There have been no reports of osteoma cutis associated with PXE. A 17-year-old Korean girl presented cutis laxa-like marked wrinkling on th e flexural area, and a skin biopsy specimen revealed multiple foci of ossif ication with irregularly clumped, basophilic-stained elastic fibers in the reticular dermis and calcium deposits along the elastic fibers. Ultrasonogr aphic evaluation showed multiple tiny osteomas diffusely scattered along th e entire abdominal wall, axillae, and medial aspect of the upper arms. We r eport the first case of osteoma cutis coexisting with cutis laxa-like PXE.