Periumbilical Perforating Pseudoxanthoma Elasticum in a Young Male Patient / 대한피부과학회지

Periumbilical perforating pseudoxanthoma elasticum is a rare acquired disorder of connective tissue. It has been suggested that repeated traumas of pregnancy, obesity, or abdominal surgery promote localized degeneration of elastic fibers. Most cases reported in the English literature occurred in obese, middle aged, multiparous women. We describe a 29-year-old male who had two hyperpigmented plaques on the periumbilical area. He had angioid streaks, but was not obese and did not have hypertension. A skin biopsy revealed transepidermal elimination of degenerated and calcified elastic fibers, which was consistent with periumbilical perforating pseudoxanthoma elasticum. We report an interesting case of periumbilical perforating pseudoxanthoma elasticum that occurred in a young man with no known predisposing risk factors.

A Case of Periumbilical Perforating Pseudoxanthoma Elasticum

Periumbilical perforating pseudoxanthoma elasticum is a rare acquired disorder that usually occurs in obese, middle-aged, multiparous women. It is considered as a separate entity from the hereditary pseudoxanthoma elasticum due to lack of hereditary transmission and association with systemic disease. A 70-year-old multiparous woman presented with a 2-year history of a gradually enlarging, yellowish ulcerated plaque in the periumbilical region. The biopsy specimen showed irregularly altered elastic fibers with encrusted calcium salts that underwent transepidermal elimination. We report a rare case of periumbilical perforating pseudoxanthoma elasticum with no signs of hereditary pseudoxanthoma elasticum.

A Case of Periumbilical Perforating Pseudoxanthoma Elasticum / 대한피부과학회지

Perforating pseudoxanthoma elasticum is a rare disorder. It is an hereditary connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. The localized lesions usually occur in a periumbilical location in obese, middle-aged, multiparous black women. It is characterized clinically by yellowish, lax, well-circumscribed, reticulated or cobblestoned patches or plaques of the periumbilical region. Herein, we report a case of periumbilical perforating pseudoxanthoma elasticum which occurred in an obese, multiparous woman with no signs of hereditary systemic pseudoxanthoma elasticum.

A Case of Perforating Pseudoxanthoma Elasticum / 대한피부과학회지

Perforating pseudoxanthoma elasticum is a localized, cutaneous disorder found mostly in obese, multiparous middle-aged women. It is characterized clinically by a slowly enlarging, yellowish, well-defined reticulated plaque usually in the periumbilical area with keratotic papules scattered on its surface, and histologically by transepidermal elimination of altered basophilic calcified elastic fibers, which are short, fragmented, and curled, in the middle portion of the reticular dermis. We present a case of a 36 year-old female who had a skin lesion, which showed chracteristic features consistent with perforating pseudoxanthoma elasticum in her neck region but not in the periumbilical area.