Pseudoxanthoma Elasticum in a young Nigerian female.

Pseudoxanthoma Elasticum is a rare inheritable disorder of elastic fibres. A 30 year old Nigerian female presented with xanthoma like papules on her neck, axillae and groin areas, deteriorating visual acuity, angioid streaks and macula mottling on fundoscopy and cardiovascular disease. Four siblings had similar skin lesions. The patient was hyperphosphataemic and hypercalcaemic. Chest x-ray showed evidence of pulmonary calcification. Skin biopsy confirmed a diagnosis of Pseudoxanthoma Elasticum. This case report amplifies the relevance of a high index of suspicion in diagnosing rare dermatologic conditions in developing countries given their often significant effects and medical importance.