Pyoderma gangrenosum - a guide to diagnosis and management

Abstract

Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. There are several subtypes, with 'classical PG' as the most common form in approximately 85% cases. This presents as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/erythematous border. The lower legs are most frequently affected although PG can present at any body site. Other subtypes include bullous, vegetative, pustular, peristomal and superficial granulomatous variants. The differential diagnosis includes all other causes of cutaneous ulceration as there are no definitive laboratory or histopathological criteria for PG. Underlying systemic conditions are found in up to 50% of cases and thus clinicians should investigate thoroughly for such conditions once a diagnosis of PG has been made. Treatment of PG remains largely anecdotal, with no national or international guidelines, and is selected according to severity and rate of progression. Despite being a well-recognised condition, there is often a failure to make an early diagnosis of PG. This diagnosis should be actively considered when assessing ulcers, as prompt treatment may avoid the complications of prolonged systemic therapy, delayed wound healing and scarring.

Keywords: Dermatology; neutrophilic dermatosis; pyoderma gangrenosum; ulcer.

Fig 1.

Rapidly progressive painful ulcer

Fig 2.

Histological findings with an intense neutrophilic infiltrate, neutrophilic pustules and abscess formation

Fig 3.

Treatment choice based on the severity and extent of pyoderma gangrenosum

Fig 4.

Biologic therapy as a treatment of pyoderma gangrenosum targeting a number of cytokines