Approach Considerations

Pyoderma gangrenosum is a diagnosis of exclusion as no specific criteria have been determined to confirm the diagnosis. All other potential causes of similar lesions must be excluded prior to making the diagnosis.

Routine blood work to evaluate for an underlying systemic illness in persons with pyoderma gangrenosum includes a complete blood count (CBC); a comprehensive chemistry profile, including a liver function test; and a urinalysis. In addition, a hepatitis profile should be performed.

Serum and/or urine protein electrophoresis, peripheral smear, and bone marrow aspiration or biopsy should be performed, if indicated, to evaluate for hematologic malignancies.

Other serum studies include a Venereal Disease Research Laboratory (VDRL) test, an antineutrophil cytoplasmic antibody test, a partial thromboplastin time test, and an antiphospholipid antibody test, all of which can help to rule out the following diseases: granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), vasculitis, and antiphospholipid antibody syndrome.

Anti–Saccharomyces cerevisiae antibodies (IgG and IgA) occur in patients with inflammatory bowel disease and might be of value for the identification of an associated disease when there are no bowel symptoms. In addition, perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) occur in some patients with inflammatory bowel disease. c-ANCA should be tested, but is not associated with inflammatory bowel disease and, in an appropriate patient, might lead to a diagnosis of granulomatous polyangiitis. Fecal calprotectin is another test to consider when assessing a patient with pyoderma gangrenosum for associated inflammatory bowel disease.

Serum immunofixation electrophoresis is helpful to determine if a monoclonal gammopathy is present. If it is, it is most frequently an IgA subtype. Myeloma, however, is a rare association.

Cultures

Tissue cultures of the ulcer/erosion for bacteria, fungi, atypical mycobacteria, and viruses are needed. The exact cultures to be performed depend on the individual situation. The cultures should be held for 6 weeks because some of the potential agents may take that long to grow in culture.

Imaging studies

Chest radiography may be performed. Angiography or Doppler studies may be carried out in patients suspected of having arterial or venous insufficiency.

Colonoscopy

Colonoscopy or other tests to exclude associated inflammatory bowel disease or ulcerative colitis may be useful in patients with symptoms. The evaluation of patients with pyoderma gangrenosum and no symptoms of bowel disease is still uncertain.

Histologic Findings

The histopathologic findings in pyoderma gangrenosum are not specific. However, a biopsy is suggested in almost all instances because it is useful in the exclusion of other diseases, such as infections and malignancy. Microscopic features include massive neutrophilic infiltration, hemorrhage, and necrosis of the overlying epidermis. Histologically, this finding may simulate an abscess or cellulitis, although no organisms are seen and cultures are generally negative or only demonstrate typical skin flora. Neutrophils are often around and within the vessel walls, but the full picture of vasculitis is generally absent.

In early disease, a mixed cell infiltrate may be present. Late in the process, granulation tissue may be present, but granuloma formation is generally believed to be incompatible with the diagnosis of pyoderma gangrenosum.

Treatment & Management

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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.