Author/Authors :
Pala, E E İzmir Tepecik Training and Research Hospital - Clinic of Pathology, Turkey , Bayol, Ü İzmir Tepecik Training and Research Hospital - Clinic of Pathology, Turkey , Usturalı Keskin, E İzmir Tepecik Training and Research Hospital - Clinic of Pathology, Turkey , Küçük, Ü İzmir Tepecik Training and Research Hospital - Clinic of Pathology, Turkey , Türe, G İzmir Tepecik Training and Research Hospital - Clinic of Ophthalmology, Turkey , Bıçakçı, C İzmir Tepecik Training and Research Hospital - Clinic of Dermatology, Turkey
Abstract :
Pseudoxanthoma elasticum (PXE) is an inherited multisystem disorder with ectopic mineralization of connective tissues and characteristic clinical manifestations in the skin, eyes and cardiovascular system. A 46-year-old woman was admitted to dermatology clinic with whitish/yellow papules that coalesce into cobblestone patterned plaques at the lateral, posterior aspect of the neck 6 previously. Two of the lesions were excised by plastic surgery. Histologically fragmented calcified elastic fibers were seen in the affected elastic tissue of the dermis accompanied by dystrophic calcinosis cutis in the superficial dermis. Fundoscopic examination revealed bilateral angioid streaks and peau d orange lesions. Because of the typical skin findings proved by biopsy and ocular involvement, our diagnosis was PXE. We should be aware of this entity since PXE is associated with considerable morbidity and mortality. The second important point is when we note calcinosis cutis, we should look for elastic tissue damage since calcium prefers depositing in those areas.
