A rare case of aggressive pyoderma gangrenosum with Cogan syndrome in a person with skin of colour.

Abstract

Cogan syndrome (CS) is a rare autoimmune vasculitis affecting the audiovestibular and ocular systems. Its pathogenesis is unknown. CS was classified into typical and atypical CS in 1980 to aid its diagnosis. Its association with pyoderma gangrenosum (PG) has only been reported three times in the literature. This is also the first case of its occurrence in a person with skin of colour. CS is a diagnosis of exclusion and thus its diagnosis may present many challenges to healthcare professionals. Herein, we describe the case of a 75-year-old South Asian woman who presented acutely to the Stroke Unit following a right lacunar infarction which was treated with aspirin and clopidogrel. An enlarging nonhealing wound was noted at the site of a recent total left hip replacement. Intravenous antibiotics were started, with multiple surgical debridements performed. During admission, two new painful pustular skin lesions erupted on the chest and abdomen that ulcerated within 2 days. Painful ulcerated lesions with bluish undermined edges were also noted at the left hip wound and two pressure areas of the buttocks. A clinical diagnosis of PG was made and treatment was started with high-dose corticosteroids, which did not lead to improvement. The patient's past medical history included left eye central retinal vein occlusion with recurrent uveitis and bilateral sensorineural deafness. A diagnosis of atypical CS was made. Four pulsed cyclophosphamide infusions and hyperbaric oxygen healed the lesions. This case demonstrates the complex interplay between PG and CS, which requires further research as it can result in significant morbidity.