Abstract
Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic dermatosis. The ulcerative subtype presents with a tender nodule or pustule that progresses into a painful, necrotic ulcer.New lesions arise after minor trauma in one-third of patients, a phenomenon termed "pathergy." We present a 62-year-old Caucasian female with primary sclerosing cholangitis, hepatic cirrhosis, chronic hepatitis B, and severe PG. At the initial presentation, she had lesions on her face and four extremities. She had severe full-thickness ulcerations on the bilateral cheeks and underwent incision and drainage with washout of bilateral maxillary abscesses, left sinus curettage, and wound debridement. She has required multiple hospitalizations for severe flares. Treatment with steroids was complicated by spinal compression fractures. Steroid-sparring agents were ineffective. Her lesions involved bilateral cheeks, temples, temporal scalp, and eyelids with oroantral fistulae. Her facial ulcerations included a large septal perforation causing saddle nose deformity and eradication of a branch of the left facial nerve causing incomplete eye closure. She underwent bilateral facial wound irrigation with antibiotic irrigation and wound debridement. Due to social factors, she has been lost to follow-up and a definitive etiology of her PG has not yet been elucidated. Although rare, PG should remain a consideration in patients with ulcerative lesions on the head and neck. Wound debridement is typically discouraged given the risk of pathergy, but there may be a role for surgical intervention in adequately immunosuppressed patients.