DOI: 10.36347/sjmcr.2018.v06i08.030

Pages: 598-602

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterized by elastorrhexia, a yellowish coalescing papules mainly on the neck and flexural areas, with ocular and cardiovascular manifestations. We report a very rare case of generalized cutis laxa-like pseudoxanthoma elasticum associated with osteoma cutis (ossification). A 26 year-old woman of a nonconsanguineous marriage consulted for generalized wrinkling of the body and multiple yellowish papules of the neck. She noticed her first manifestations at the age of 16 years. She has no similar diseases in her family and never had autoimmune, inflammatory, cardiac or ocular diseases as a preceding history. A skin biopsy showed ossification and fragmentation of elastic fibers in the dermis. A medical, histological and laboratory workup was initiated showing no abnormal changes and organs involvement. A pseudoxanthoma elasticum with generalized cutis laxa-like disorder, as well as an extremely rare skin ossification (Osteoma cutis) was our case very interesting discovery. No other organ involvement was discovered which suggests a careful follow-up of possible complications.