PERIUMBILICAL PERFORATING PSEUDOXANTHOMA ELASTICUM: A RARE CASE REPORT WITH A DISTINCTIVE PRESENTATION

Periumbilical perforating pseudoxanthoma elasticum (PPPXE) presents as well-defined, yellowish, atrophic plaques encircling keratotic papules, often observed in obese, multiparous women. This dermatosis is considered either a localized acquired cutaneous disorder or a variant of hereditary pseudoxanthoma elasticum. We report a case clinically diagnosed with differentials but confirmed as PPPXE through histopathological examination. The patient, a middle-aged woman, presented with characteristic lesions in the periumbilical region, prompting consideration of various differential diagnoses. However, microscopic analysis revealed the distinctive features of perforating pseudoxanthoma elasticum, including the presence of transepidermal elimination channels and abnormal elastic fibers. This case underscores the importance of histopathological evaluation in confirming the diagnosis of PPPXE, especially when clinical presentation overlaps with other cutaneous conditions. Awareness of this entity is crucial for accurate diagnosis and appropriate management, given its potential implications for systemic health. Further research is warranted to elucidate the pathogenesis and optimal treatment strategies for PPPXE. Our case contributes to the expanding literature on this rare dermatosis, emphasizing the significance of a multidisciplinary approach involving dermatologists and pathologists for its diagnosis and management.