A Case of Periumbilical Perforating Pseudoxanthoma Elasticum: CASE REPORT

A 70-year-old obese multiparous woman presented with a 2-year history of gradually enlarging ulcerated plaques on her periumbilical region. On physical examination of the skin lesion, there were two discrete, 0.7 x 0.5 cm and 1.0 x 0.8 cm, yellowish to brownish cobblestoned plaques with central ulcer­ation in the supraumbilical area (Fig. 1A). She had not any history of abdominal surgery or trauma on the abdomen but had a history of 5 vaginal deliveries. Her other medical history was un­remarkable. On examination of her body, there were no abnormal findings, including in her cardiovascular and ophthalmologic systems. Laboratory evaluations including complete blood count, blood chemistry analysis and urinalysis were normal.

A punch biopsy was taken from the yellowish ulcerated plaque on the supraumbilical area. Histopathologically, the epidermis showed a keratin-filled crater. The upper dermis demonstrated irregularly altered elastic fibers with encrusted calcium salts extruded to the surface through the epidermis in a wide channel, that is, transepidermal elimination (Fig. IB). The Verhoeff- van Gieson stain showed numerous clumped, ir­regularly thickened elastic fibers in the dermis (Fig. 1С). The von Kossa stain revealed fragmented and calcified elastic fibers in the dermis (Fig. 1D). These clinical and histopathologic findings were consistent with those of PPPXE. Because there was no known effective therapy, we assured the patient that this was a benign disorder and decided to keep her on close observation. There has not been any pro­gression or change of the lesion during the 8-month follow-up period.
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Fig. 1. (A) Ill-defined yellowish to brownish cobblestoned plaques with central ulceration were noted in the supraumbilical region. (B) Transepidermal elimination of altered elastic fibers were seen in the dermis (H & E, x 40). (C) Clumped, irregularly thickened elastic fibers were seen in the dermis (Verhoeff-van Gieson stain, * 100). (D) Calcified and fragmented elastic fibers were demonstrated (von Kossa stain, x 100).


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