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Case Reports

Abstract

Pseudoxanthoma elasticum (PXE) is an inherited genodermatosis that causes extensive degeneration of elastic tissue due to abnormal calcification. There are several published cases that describe PXE-like histological changes in a number of inflammatory conditions without overt clinical manifestation of PXE. Such reported cases include lipodermatosclerosis, granuloma annulare, lichen sclerosus, morphea profunda, erythema nodosum, septal panniculitis, and fibrosing dermatitis, as well as basal cell carcinoma. Herein, we present a case of peristomal pyoderma gangrenosum (PG) with histological features reminiscent of PXE. The patient was a 64-year-old female with a medical history significant for ulcerative pancolitis treated with infliximab and ostomy, atrial fibrillation, and stage III chronic kidney disease (CKD) who presented to the emergency department with high ostomy output. On the third day of admission, dermatology was consulted due to concern for desquamating rash. Physical examination revealed multiple peristomal ulcers. Biopsy revealed focal ulceration of the epidermis with a fibroinflammatory crust.  In the dermis, a diffuse mixed inflammatory infiltrate composed of lymphocytes, histiocytes and neutrophils with karryorrhectic debris was observed. Also identified in the dermis were basophilic and fragmented elastic fibers, reminiscent of those seen in PXE. A von Kossa stain demonstrated calcified elastic fibers in the dermis. The patient responded promptly to systemic corticosteroids. Utilizing Delphi criteria, a diagnosis of peristomal pyoderma gangrenosum with PXE-like histologic features was made. To our knowledge, this report is the first to describe a case of PG in association with PXE-like histologic features.