ABSTRACT
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.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Pregnancy , Angioid Streaks , Biopsy , Connective Tissue , Elastic Tissue , Hypertension , Obesity , Pseudoxanthoma Elasticum , Risk Factors , SkinABSTRACT
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.