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Report of a Case in a Patient With Cystic Fibrosis

Jeffrey B. Tiger, BA; Jill McKenzie, MD; Don T. Tran, MD; John E. Olerud, MD; Evan George, MD

Arch Dermatol. 2009;145(11):1292-1295.

Background  There is scant literature that documents pseudoxanthoma elasticum (PXE)–like histologic changes in the setting of inflammatory skin diseases. This article documents granulomatous dermatitis with PXE-like changes in a patient with cystic fibrosis. This is the first report of its kind, to our knowledge.

Observations  A 33-year-old woman with cystic fibrosis developed a papular eruption on the flexural surfaces of the upper and lower extremities, which was initially treated with prednisone. A punch biopsy showed granulomatous inflammation and associated PXE-like changes. The combined histologic and clinical findings were most consistent with granuloma annulare. There was no family history of PXE or clinical manifestations of PXE. The rash gradually resolved itself over the next several months.

Conclusions  There are few publications that document PXE-like changes in association with various inflammatory skin conditions. Thus, the clinical significance of this finding remains uncertain. This case and previous reports are discussed in the context of current molecular and genetic knowledge. It is hoped that greater awareness of this phenomenon will promote further investigation and elucidation of the clinical and biologic significance of PXE-like changes observed in biopsies of inflammatory skin disorders.

Author Affiliations: Departments of Dermatology (Drs McKenzie and Olerud) and Anatomic Pathology (Dr George) and School of Medicine (Mr Tiger), University of Washington, Medical Center, Seattle; and Cellnetix Pathology and Laboratories, Seattle (Dr Tran).

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