Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks - Apparent regression with hemodialysis

A. N. Sapadin, M. G. Lebwohl, S. A. Teich, R. G. Phelps, D. DiCostanzo, S. R. Cohen

Research output: Contribution to journalArticlepeer-review

42 Scopus citations

Abstract

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneous involvement is most commonly seen in the rare entity termed periumbilical perforating pseudoxanthoma elasticum (PPPXE). Patients with this disorder are generally obese, middle aged, multiparous black women with hypertension. The cutaneous lesions are well-demarcated, hyperpigmented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described. We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrated typical calcification of elastic fibers with additional amorphous calcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions - both clinically and histopathologically. The relation between PPPXE and hereditary PXE is discussed. The role of chronic renal failure in precipitating PPPXE is considered.

Original languageEnglish (US)
Pages (from-to)338-344
Number of pages7
JournalJournal of the American Academy of Dermatology
Volume39
Issue number2 II
StatePublished - Jan 1 1998
Externally publishedYes

ASJC Scopus subject areas

  • Dermatology

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