Yellowish periumbilical plaque with keratotic papules

A 58-year-old obese, hypertensive, female presented with itchy yellowish papules coalescing to form a reticulated plaque surrounding the umbilicus circumferentially, for the past 7 months [Figure 1]. There were a few erythematous papules with central keratotic plug at the periphery, some of which had healed with round-oval depressed atrophic scars [Figure 1inset].

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Figure 1:

Yellowish papules coalescing to form a plaque around the umbilicus, with a few erythematous papules and scarring at the periphery. (Inset view) Erythematous keratotic papules, healing with depressed round-oval scars

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She was a multiparous woman with two children and had undergone laparoscopic tubal ligation 15 years back. Her family history was unremarkable. An electrocardiogram, two-dimensional-echocardiograph and ophthalmological examination were within normal limits. Skin biopsy from the yellowish plaque and keratotic papule showed fragmented, curly elastic fibers in the reticular dermis [Figure 2a] and transepidermal elimination of degenerated elastic fibers [Figure 2b], respectively.

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Figure 2 a:

Histopathology from the yellowish plaque shows fragmented, curly elastin fibers in the reticular dermis (H and E, 200×)

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Figure 2 b:

Biopsy of the keratotic papule shows transepidermal elimination of degenerated elastic fibres. (H and E, 40×)

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Periumbilical perforating pseudoxanthoma elasticum is widely regarded as a localized and acquired form of hereditary pseudoxanthoma elasticum, lacking the systemic involvement. Our patient was obese and multiparous, possible risk factors for perforating pseudoxanthoma elasticum. Her itching improved and keratotic papules flattened with oral anti histamines and clobetasol propionate 0.05% ointment local application.