Clinical features, causes, treatments, and outcomes of peristomal pyoderma gangrenosum (PPG) in 44 patients: The Mayo Clinic experience, 1996 through 2013

J Am Acad Dermatol. 2016 Nov;75(5):931-939. doi: 10.1016/j.jaad.2016.05.044. Epub 2016 Jul 26.

Abstract

Background: There are limited large case series of peristomal pyoderma gangrenosum (PPG), an uncommon cause of recalcitrant peristomal ulceration.

Objective: We sought to further characterize the clinical features, causes, treatments, and outcomes of PPG.

Methods: We conducted a retrospective chart review of patients with PPG seen at Mayo Clinic from January 1996 to July 2013.

Results: A total of 44 patients had PPG (mean age, 46 years; 32 women [73%]); 41 (93%) had inflammatory bowel disease. Mean time to PPG onset after stoma surgery was 5.2 months (excluding 1 outlier). Systemic therapies included corticosteroids (66%), immunosuppressants (41%), biologics (36%), and a combination of systemic treatments (36%). Mean time to reach a complete response was 10.7 weeks. Stoma closure had the greatest complete response (4 of 4 patients, no recurrences). Recurrence after any treatment was documented in 23 of 38 (61%) patients. Stoma relocation/revision recurred in 10 of 15 (67%) patients. Remission occurred in 29 of 31 (94%) patients.

Limitations: Small sample size and retrospective study design are limitations.

Conclusion: PPG is strongly associated with inflammatory bowel disease, is predominant in women, and has a prolonged time to onset and high recurrence rate. Systemic corticosteroid or combination therapies and surgical closure can be effective treatments. Timely recognition and management are paramount to achieving early remission.

Keywords: Crohn's disease; inflammatory bowel disease; neutrophilic dermatosis; pyoderma gangrenosum; stoma; ulcer.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Aged, 80 and over
  • Anti-Bacterial Agents / therapeutic use
  • Biological Products / adverse effects
  • Biological Products / therapeutic use
  • Diagnostic Errors
  • Disease Susceptibility
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunocompromised Host
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / adverse effects
  • Inflammatory Bowel Diseases / surgery
  • Male
  • Middle Aged
  • Postoperative Complications / drug therapy
  • Postoperative Complications / epidemiology
  • Postoperative Complications / etiology*
  • Postoperative Complications / surgery
  • Pyoderma Gangrenosum / diagnosis
  • Pyoderma Gangrenosum / drug therapy
  • Pyoderma Gangrenosum / epidemiology
  • Pyoderma Gangrenosum / etiology*
  • Pyoderma Gangrenosum / surgery
  • Recurrence
  • Reoperation
  • Retrospective Studies
  • Risk Factors
  • Skin Ulcer / drug therapy
  • Skin Ulcer / epidemiology
  • Skin Ulcer / etiology
  • Surgical Stomas / adverse effects*
  • Young Adult

Substances

  • Adrenal Cortex Hormones
  • Anti-Bacterial Agents
  • Biological Products
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents