Periumbilical perforating pseudoxanthoma elasticum: a rare case report

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.

Two cases of periumbilical perforating pseudoxanthoma elasticum with dermoscopic and histopathologic features

“…They describe these findings as being nonspecific and have not described keratotic papules or curvilinear hyperpigmented lines. The latter have only been noted by Jha et al but have not been described in detail. This has been done by Vishwanath et al in a case of long‐standing nonperforating periumbilical PXE wherein only hyperpigmented arcuate lines were noted.…”

“…Three reports detail dermoscopic features of periumbilical perforating PXE . Jha et al describe keratotic plugs (correlating with transepidermal elimination of altered elastic fibres on histopathology) and telangiectasias, dotted vessels and erythema (correlating with upper dermal inflammation and capillary dilatation on histopathology). Nasca et al have described orange‐yellow pigmentation with white and red areas and microulcerations in a case of perforating PXE on the neck.…”

“…Reports describing treatment are sparse owing to the rarity of this disease and no cure exists. Treatment modalities include dietary calcium restriction (less than 800mg per day) and oral phosphate binders . Plastic surgery has been described as a means to improve appearance …”

Periumbilical Papules in a Middle-aged Woman

“…5 Angioid streaks are noted in 22% of cases. 5 Histopathologic features are acanthosis, pseudoepitheliomatous hyperplasia, and irregularly fragmented elastic fibers at varying levels in the upper reticular and midreticular dermis, 6 which are extruded. These elastic fibers assume a ravelled wool Two cases are reported wherein no perforation was noted despite a duration of 2 to 3 years (as in this case).…”

“…Histopathologic features are acanthosis, pseudoepitheliomatous hyperplasia, and irregularly fragmented elastic fibers at varying levels in the upper reticular and midreticular dermis, which are extruded. These elastic fibers assume a ravelled wool appearance—they are clumped, crinkly, and eosinophilic with calcium deposits.…”