Acquired pseudoxanthoma elasticum

Acquired pseudoxanthoma elasticum (acquired PXE), also known as perforating calcific elastosis or periumbilical perforating pseudoxanthoma elasticum, is a rare condition that mimics the skin findings of hereditary pseudoxanthoma elasticum (PXE) but occurs without an underlying genetic mutation in the ABCC6 gene. Unlike the inherited form, acquired PXE develops later in life and is typically localized to specific areas of the skin, most commonly the periumbilical region (around the belly button), though other sites can be affected. The condition is characterized by yellowish papules and plaques with a cobblestone-like or plucked-chicken-skin appearance, resulting from calcification and fragmentation of elastic fibers in the mid-dermis. Acquired PXE has been associated with various predisposing factors, including obesity, prior abdominal surgery, pregnancy (particularly multiple pregnancies), and conditions that cause increased abdominal pressure or local trauma. Unlike hereditary PXE, acquired PXE generally does not involve systemic complications such as cardiovascular disease, gastrointestinal bleeding, or the characteristic angioid streaks of the retina, although careful ophthalmologic and cardiovascular evaluation is recommended to exclude the inherited form. There is no specific curative treatment for acquired PXE. Management is primarily supportive and focuses on addressing any underlying contributing factors, such as weight management and avoidance of further mechanical stress to affected areas. The condition is generally considered benign and localized, with a much more favorable prognosis compared to hereditary PXE. Dermatologic follow-up is recommended, and genetic testing may be pursued to definitively distinguish acquired PXE from the hereditary form.

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