Abstract
Periumbilical perforating pseudoxanthoma elasticum (PPPXE) usually presents with well-defined periumbilical yellowish atrophic plaques with keratotic papules at the periphery. It is considered a variant of hereditary pseudoxanthoma elasticum or a localized acquired cutaneous dermatosis. The lesions usually occur in the periumbilical area in obese, multiparous women. Here, we report an additional case of periumbilical perforating pseudoxanthoma elasticum with its dermoscopic features.
Highlights
Pseudoxanthoma elasticum (PXE) is a rare inherited disease of connective tissue causing fragmentation and mineralization of elastic fibers that primarily affects the skin, retina, and cardiovascular system [1]
Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is considered a localized variant of inherited PXE based on the presence of angioid streaks (22% of cases) and flexural lesions [3]
Some believe it to be an acquired dermatosis secondary to cutaneous trauma caused by multiple pregnancies, obesity, and multiple abdominal surgeries or trauma resulting in elastic fiber degeneration in these patients [4,5]
Summary
Pseudoxanthoma elasticum (PXE) is a rare inherited disease of connective tissue causing fragmentation and mineralization of elastic fibers that primarily affects the skin, retina, and cardiovascular system [1]. Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is considered a localized variant of inherited PXE based on the presence of angioid streaks (22% of cases) and flexural lesions [3]. Some believe it to be an acquired dermatosis secondary to cutaneous trauma caused by multiple pregnancies, obesity, and multiple abdominal surgeries or trauma resulting in elastic fiber degeneration in these patients [4,5]. We report on a case of acquired PPPXE with dermoscopic features
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