Pyoderma Gangrenosum in a 10-year-old boy
Henry Foong FRCP
Dec 31, 2006
Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia
Skin Ulcers Misdiagnosed as Pyoderma Gangrenosum. Roger H. Weenig, M.D., Mark D.P. Davis, M.D., Patrick R. Dahl, M.D., and W.P. Daniel Su, M.D. (NEJM 2002; 347: 1412-1418)
Mayo Clinic, Minnesota, USA
Background Pyoderma gangrenosum is a diagnosis of exclusion, and the misdiagnosis of pyoderma gangrenosum can result in substantial complications in patients who have other causes of severe cutaneous ulceration.
Methods We reviewed the charts of 240 patients with a diagnosis of pyoderma gangrenosum who were evaluated at our institution from 1975 through 2000, including 157 consecutive patients treated for presumed pyoderma gangrenosum from 1984 through 1992. We also reviewed the English-language literature.
Results Ninety-five patients (49 from our institution and 46 described in the literature) had skin ulcers with a clinical resemblance to pyoderma gangrenosum. The final diagnoses were vascular occlusive or venous disease, vasculitis, cancer, primary infection, drug-induced or exogenous tissue injury, and other inflammatory disorders. Of the 95 patients studied, 64 had been treated for pyoderma gangrenosum for a median of 10 months (range, 3 to 180). These 64 included 15 of the 157 consecutive patients treated for pyoderma gangrenosum at our institution (10 percent). Of the ulcers in the 64 patients treated for pyoderma gangrenosum, it was clear that those in 23 patients (36 percent) did not respond to treatment directed at pyoderma gangrenosum, those in 8 (12 percent) were exacerbated by such treatment, and those in 15 (23 percent) improved with such treatment.
Conclusions The misdiagnosis of pyoderma gangrenosum is not uncommon and exposes patients to risks associated with its treatment. A thorough evaluation is required in all patients suspected of having pyoderma gangrenosum in order to rule out alternative diagnoses.
Pyoderma gangrenosum: clinicopathologic correlation and proposed
Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.
Infliximab for the treatment of pyoderma gangrenosum: a randomised,
double-blind placebo-controlled trial Trevor N Brooklyn
1, Giles S Dunnill 1, Ajeya Shetty 2, June J Bowden 3, Jason DL Williams
3, Christopher EM Griffiths 3, Alastair EM Forbes 2, Rosemary Greenwood
1 and Chris S Probert 1 (Gut. 2006 Apr;55(4):505-9)
Background: Pyoderma gangrenosum (PG) is a chronic ulcerating skin condition that often occurs in association with inflammatory bowel disease (IBD). There have been a number of reports of PG responding to infliximab, a monoclonal antibody against tumour necrosis factor alpha (TNF).
Aim: In the first randomised placebo-controlled trial of any drug for the treatment of PG, we have studied the role of infliximab in this disorder.
Subjects: Patients 18 years of age or older with a clinical diagnosis of PG were invited to take part.
Methods: Patients were randomised to receive an infusion of infliximab at 5mg/kg or placebo at week 0. Patients ere then assessed at week 2 and non-responders were offered open-labelled infliximab. The primary end- point was clinical improvement at week 2, with secondary end-points being remission and improvement at week 6.
Results: 30 patients were entered into the study. After randomization, 13 patients received infliximab and 17 patients received placebo. At week 2, significantly more patients in the infliximab group had improved 46% (6/13) as compared with the placebo group 6% (1/17), p = 0.025. Overall, 29 patients received infliximab with 69% (20/29) demonstrating a beneficial clinical response. The remission rate at week 6 was 21% (6/29). There was no response in 31% (9/29) of patients.
Conclusions: This study has demonstrated that infliximab at a dose of 5mg/kg is superior to placebo in the treatment of PG. Open label treatment with infliximab also produced promising results. Infliximab treatment should be considered in patients with PG.
Treatment recommendations for pyoderma gangrenosum: An evidence-based
review of the literature based on more than 350 patients
(J Am Acad Dermatol 2005; 53: 273-283.)
Because the incidence of pyoderma gangrenosum (PG) is low, no prospective randomized controlled trials and only a few studies with case numbers of more than 15 patients have been published. To date no guidelines for treatment of PG have been established far.
The aim of the study was to provide an evidence-based review of the literature and an evaluation of recommendations for PG treatment. We performed an electronic search using the PubMed database and the term “pyoderma-gangrenosum.” Literature published in the English language during the past two decades was reviewed. All relevant studies that could be obtained regardless of the study design were evaluated for grades of recommendation and levels of evidence. Data on patient characteristics including severity of the disease, localization of lesions, associated diseases, and treatment procedures were abstracted and evaluated for therapeutic outcome.
We conclude that therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented in the literature for disseminated as well as for localized disease and should be considered first-line therapy. In cases that do not respond to this treatment, we recommend alternative therapeutic procedures (eg, systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; infliximab; or plasmapheresis), considering additional factors including associated diseases.