Clinicopathological Profile of Pyoderma Gangrenosum: A 10-Year Retrospective Study from a Tertiary Care Center in South India

Abstract

Background: Pyoderma gangrenosum (PG) is a reactive neutrophilic inflammatory dermatosis with a varied clinicopathologic presentation. It commonly manifests as rapidly progressive painful ulcers, mimicking varied conditions including infections, vasculitis, and malignancies, and is a diagnosis of exclusion. There are scarce data on PG from the Indian subcontinent. Aim and Objectives: The aim of the study was to study the clinicopathologic profile of patients with PG and their underlying systemic associations. Materials and Methods: A retrospective observational study was done between 2011 and 2021, and patients diagnosed as PG based on the diagnostic tool proposed by Maverakis et al. were recruited and their demographic, clinical, and histological findings were obtained. Results: Among 54 patients with suspected PG, 17 patients (eight males and nine females) fulfilled the diagnostic criteria, and the mean age of disease onset was 32.1 years (range: 3–60 years). Ulcerative variant was the most common type (9/17, 52.9%), and 29.4% had systemic associations including autoinflammatory syndromes. The onset at atypical sites such as face and hand were noted in one patient each. Histopathology revealed a polymorphous dermal infiltrate with neutrophilic predominance in the majority (94.1%). Systemic steroids (dose ranging from 0.5–1 mg/kg prednisolone equivalent) were used in 11/17 (64.7%) patients. The commonly used alternative drugs included clofazimine (47%), minocycline (29%), thalidomide (23.5%), adalimumab and mycophenolate mofetil in 17.6% each, dapsone and ciclosporine in 11.7% each. Remission was achieved between two weeks and three months in 10 (58.8%) patients after treatment initiation and two mortalities (11.7%) were recorded. Conclusion: PG can affect any age group and may be localized to rarer, atypical sites. The possibility of underlying autoinflammatory conditions should be considered in addition to the evaluation of other disorders like inflammatory bowel disease, hematological disorders, and rheumatological disorders.

Keywords

• autoinflammatory syndromes
• neutrophilic dermatosis
• pyoderma gangrenosum