From the Guidelines
Pyoderma gangrenosum typically presents as a painful, rapidly evolving ulcer with a characteristic violaceous, undermined border, often beginning as a pustule, nodule, or inflammatory papule that breaks down to form an ulcer with a necrotic base, as described in the most recent and highest quality study 1. The lesions most commonly occur on the lower extremities, particularly the pretibial area, but can appear anywhere on the body including the trunk, head and neck, and peristomal areas, with a reported incidence of 0.6–2.1% in UC patients 1. Some key characteristics of pyoderma gangrenosum include:
- Pain out of proportion to the clinical appearance
- Minor trauma preceding the development of lesions (pathergy)
- Systemic symptoms such as fever, malaise, and arthralgia
- Association with underlying systemic diseases in 50-70% of cases, particularly inflammatory bowel disease, rheumatoid arthritis, and hematologic malignancies Diagnosis is primarily clinical and requires exclusion of other causes of ulceration, as there are no specific laboratory or histopathologic findings, and biopsy of the ulcer edge may help rule out other conditions but should be performed cautiously to avoid triggering new lesions due to pathergy 1. The therapeutic goal should be rapid healing, as pyoderma gangrenosum can be a debilitating skin disorder, and treatment options include systemic corticosteroids, infliximab, or adalimumab, with infliximab showing significant improvement in patients with pyoderma gangrenosum, particularly those with short duration of the disease 1.
From the Research
Clinical Presentation of Pyoderma Gangrenosum
- Pyoderma gangrenosum (PG) is a rare, neutrophil-mediated, auto-inflammatory dermatosis characterized by painful, necrotic ulceration 2, 3.
- It typically affects patients in the third to sixth decades of life, with almost equal incidence in men and women 3.
- PG occurs most frequently on the lower extremities 3.
- Five clinical variants are currently recognized: classic, bullous, pustular, vegetative, and peristomal types 3.
Associated Conditions and Diagnostic Challenges
- Half of PG cases are seen in association with systemic disease 3.
- Mimickers include infection, vascular insufficiency ulcers, systemic vasculitides, autoimmune disease, cancer, and exogenous tissue injury, among others 3.
- PG is often a diagnosis of exclusion, as there are no specific laboratory or histopathologic findings to confirm the diagnosis 3.
- A high index of suspicion is necessary for early and accurate diagnosis and prompt treatment 2.
Treatment Options and Management
- Successful management of PG typically requires multiple modalities to reduce inflammation and optimize wound healing, in addition to treatment of any underlying diseases 3.
- 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-α inhibitors, for refractory cases of PG 3, 4.
- Topical treatment of PG has gained attention in recent years, with several reports showing improvement of cutaneous lesions following topical treatment 5.
- Wound management includes gentle cleansing without sharp debridement, limited topical antibacterial use, and maintenance of a moist environment to promote epithelial migration 6.