A Case Report of Pseudoxama Elasticum of the Neck: Surgical Excision and The Effect of Laserbrasion

ABSTRACT

Pseudoxanthoma elasticum (PXE) is an uncommon heritable disorder of elastin characterized by cutaneous, ocular and vascular abnormalities. Typical skin lesion shows yellowish papules and redundant skin folds on the neck, axilla and groin due to degeneration and calcification of elastic fibers in deep dermis. As in all heritable disorders of connective tissue, no curative therapy is presently know. Redundant and unsightly skin folds about the neck can be improved by surgery, but irregular papular lesion on skin does not improved by surgery. A-44-year-old female who had a skin lesion and angioid streak was treated as follows Skin folds on the neck were excised successfully without any significant problem such as intraoperative bleeding, wound dehiscence or hypertrophic scar formation. To evaluate whether we could smooth the irregular skin surface due to multiple papules, laserbrasion with a SLT contack NdYAG laser system was performed experimentally on a limited area which was located on the right supracla-vicular area about 2 x 3cm in size. An acceptable aesthetic appearance was achieved with surgical excision 12 months after the operation. Biopsy of the laserbrasion site revealed disappearance of the calcium deposit. This study emphasizes the possibility of using laserbrasion in solving troblesome skin lesion of the PXE.