Etiology and Management of Pyoderma Gangrenosum A Comprehensive Review

• Published in: American journal of clinical dermatology Vol. 13; no. 3; pp. 191 - 211
• Main Authors: Ahronowitz, Iris, Harp, Joanna, Shinkai, Kanade
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.06.2012; Adis International
• Subjects: Adrenal Cortex Hormones - therapeutic use; Anti-Bacterial Agents - therapeutic use; Anti-Inflammatory Agents - therapeutic use; Arthritis - complications; Biological and medical sciences; Biological Products - therapeutic use; Dermatology; Hematologic Diseases - complications; Humans; Immunosuppressive Agents - therapeutic use; Inflammatory Bowel Diseases - complications; Medical sciences; Medicine; Medicine & Public Health; Pharmacology/Toxicology; Pharmacotherapy; Pyoderma Gangrenosum - complications; Pyoderma Gangrenosum - diagnosis; Pyoderma Gangrenosum - etiology; Pyoderma Gangrenosum - therapy; Review Article; Skin involvement in other diseases. Miscellaneous. General aspects; Ustekinumab; Crohn Disease; Hidradenitis Suppurativa; Inflammatory Bowel Disease; Pyoderma Gangrenosum; Skin disease; Clinical management; Neutrophilic dermatosis; Dermatology; Etiology; Bibliographic review; Pyoderma gangrenosum
• ISSN: 1175-0561; 1179-1888
• DOI: 10.2165/11595240-000000000-00000

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful, necrotic ulceration. It typically affects patients in the third to sixth decades of life, with almost equal incidence in men and women. PG occurs most frequently on the lower extremities. Five clinical variants are currently recognized: classic, bullous, pustular, vegetative, and peristomal types. Half of PG cases are seen in association with systemic disease. Mimickers include infection, vascular insufficiency ulcers, systemic vasculitides, autoimmune disease, cancer, and exogenous tissue injury, among others. PG is often a diagnosis of exclusion, as there are no specific laboratory or histopathologic findings to confirm the diagnosis. PG thus presents many clinical challenges: it is difficult to diagnose, is frequently misdiagnosed, and often requires a work-up for underlying systemic disease. Successful management of PG typically requires multiple modalities to reduce inflammation and optimize wound healing, in addition to treatment of any underlying diseases. Prednisone and cyclosporine have been mainstays of systemic treatment for PG, although increasing evidence supports the use of biologic therapies, such as tumor necrosis factor-a inhibitors, for refractory cases of PG. Here, we review the clinical presentation and pathophysiology of PG, as well as its associated conditions, diagnostic work-up, and management.