Clinical Review
Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients

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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.

Section snippets

Method

An electronic search using the PubMed database was performed using the term “pyoderma-gangrenosum.” Literature published in the English language at least in the past two decades was reviewed. In addition, pertinent references from articles and books not identified by search engines and relevant textbooks were analyzed.

Ulcers mimicking PG

Diseases with cutaneous manifestations resembling PG, including Wegener's granulomatosis, polyarteritis nodosa, lymphoma, sporotrichosis, and antiphospholipid syndrome, may lead to the misdiagnosis of PG.10 Therefore these diseases have to be excluded before diagnosing and treating PG.

Associated diseases

Fifty percent to 70% of PG cases are associated with systemic diseases. It has been demonstrated that both type and severity of associated disorders are of high importance for the prognosis of PG. Frequently,

General conclusions

The introduction of new treatment modalities has improved and broadened therapeutical options for PG. However, it must be emphasized that more rigorous comparative trials are urgently needed for definitive conclusions with respect to PG treatment. Because no prospective double-blind studies have been published and case numbers of other published studies are very low, recommendations have to be interpreted with great care.

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