Abstract

Periumbilical perforating pseudoxanthoma elasticum is a rare perforating dermatosis characterized by transepidermal elimination of abnormal elastic fibers with calcium deposition. It typically affects obese, middle-aged, multiparous women and presents as hyperpigmented plaques around the umbilicus. Although often limited to the skin, PPPXE may be associated with ocular and cardiovascular involvement. Fewer than 50 cases have been reported in the literature. We report a case of a 65-year-old multiparous woman with a 6-year history of pruritic abdominal lesions unresponsive to antifungals and corticosteroids. Clinical examination revealed irregular, atrophic scars with keratotic papules. Dermoscopy showed yellowish-brown structureless areas with central keratotic plugs. Histopathology demonstrated epidermal hyperplasia, hypergranulosis, parakeratotic columns, and transepidermal elimination of fragmented elastic fibers. Ophthalmologic evaluation revealed retinal pigment epithelium mottling. The patient had a history of hypertension but no family history of similar lesions. Etiology of PPPXE is not well established with some researchers considering it a localised cutaneous variant of pseudoxanthoma elasticum while others consider it a distinct clinical entity. This case highlights the rarity of PPPXE and underscores the importance of systemic evaluation, particularly ocular assessment, in suspected cases.