Approach Considerations

No specific therapy is uniformly effective for patients with pyoderma gangrenosum. In patients with an associated, underlying disease, effective therapy for the associated condition may be linked to a control of the cutaneous process as well.

Topical therapies include gentle local wound care and dressings, superpotent topical corticosteroids,^[20]cromolyn sodium 2% solution, nitrogen mustard, and 5-aminosalicylic acid. The topical immune modifiers tacrolimus and pimecrolimus may have some benefit in certain patients.^[21]

Systemic therapies include corticosteroids, cyclosporine,^{[22, 23, 24]}mycophenolate mofetil,^{[25, 26, 27]}azathioprine,^[28]dapsone, tacrolimus, cyclophosphamide, chlorambucil,^[29]thalidomide, tumor necrosis factor-alpha (TNF-alpha) inhibitors (eg, thalidomide, etanercept, infliximab, adalimumab,^[30]clofazimine^[31]), and nicotine.

Intravenous (IV) therapies include pulsed methylprednisolone,^[32]pulsed cyclophosphamide,^[33]infliximab,^{[34, 35, 36, 37]}IV immunoglobulin,^[38]and ustekinumab.^[39]Other therapies include hyperbaric oxygen.^[40]

Canakinumab proved effective in a patient with concomitant hidradenitis suppurativa.^[41]

Other new biologic agents are currently in early trials and may also be helpful for treating pyoderma gangrenosum or other inflammatory conditions. These include the interleukin 23, phosphodiesterase 4 inhibitors, along with the newer Janus kinase inhibitors and intravenous immune globulin. All have case reports demonstrating benefit.^{[42, 43, 44]}

Surgery

Surgery should be avoided, if possible, because of the pathergic phenomenon that may occur with surgical manipulation or grafting, resulting in wound enlargement.^[45]Pathergy is seen in about 30% of cases. In some patients, grafting has resulted in the development of pyoderma gangrenosum at the harvest site. In cases in which surgery or superficial debridement is required, the best plan, if possible, is to have the patient on therapy, and active disease under control, in order to prevent the development of new pyoderma gangrenosum lesions.^[46]

Some patients with ulcerative colitis have responded to total colectomy; in other patients, however, the disease is peristomal and occurs following bowel resection.

Transfer

Care of the patient with pyoderma gangrenosum is often referred from the general dermatologist to tertiary centers where such patients are seen more frequently.

Follow-up

Patients with pyoderma gangrenosum should receive follow-up care on a regular basis to monitor drug therapy and to measure the size of the lesion or lesions. Multiple methods of wound care are available.

Activity

Patients should maintain their range of motion and perform all activities that they are able to tolerate.

Consultations

Working with the primary care physician is wise for all patients. Depending on patient findings, however, other specialists may need to be consulted, including the following:

Gastroenterologist or GI surgeon, proctorectal surgeon, or general surgeon - For patients with inflammatory bowel disease
Rheumatologist - For patients with arthritis
Ophthalmologist - If ocular disease is present
Hematologist/oncologist - When preleukemia, leukemia, monoclonal gammopathy, or other neoplasm is associated
Plastic surgeon or general surgeon - When debridement or grafting is deemed necessary, but again should be performed with caution or while on appropriate therapy, to prevent the potential for pathergy

Medication

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Media Gallery

Classic, or typical, pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.
Peristomal pyoderma gangrenosum.
Factitial ulceration on the scalp from chronic manipulation mimicking an ulceration of pyoderma gangrenosum.

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Author

J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Acknowledgements

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.