History

Patients with pyoderma gangrenosum usually describe the initial lesion as a bite reaction, with a small, red papule or pustule changing into a larger, ulcerative lesion. Others may present with cellulitis or what they think is an abscess. Often, patients give a history of a brown recluse or other spider bite, but they have no evidence that a spider actually caused the initial event.

Pain is the predominant historical complaint. Arthralgias and malaise are often present.

A complete history should be taken with special focus on the organ systems discussed below to determine any underlying systemic disease. Systemic illnesses are seen in 50% of patients with pyoderma gangrenosum and may occur prior to, concurrently with, or following the diagnosis.

Commonly associated diseases include inflammatory bowel disease,^[8]either ulcerative colitis or regional enteritis/Crohn's disease, and a polyarthritis that is usually symmetrical and may be either seronegative or seropositive. Hematologic diseases/disorders are other commonly associated conditions; these include leukemia or preleukemic states, predominantly myelocytic in nature or monoclonal gammopathies (primarily immunoglobulin A [IgA]).^{[8, 12, 13]}

Less commonly associated diseases include other forms of arthritis, such as psoriatic arthritis, osteoarthritis, and spondyloarthropathy; hepatic diseases, including hepatitis and primary biliary cirrhosis; myelomas (IgA type predominantly); and immunologic diseases, such as lupus erythematosus and Sjögren syndrome.^{[14, 15]}

Physical Examination

Classic pyoderma gangrenosum, as shown in the image below, is characterized by a deep ulceration with a violaceous border that overhangs the ulcer bed. These lesions of pyoderma gangrenosum most commonly occur on the legs, but they may occur anywhere on the body.

Classic, or typical, pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.

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Classic pyoderma gangrenosum may occur around stoma sites; this type, shown in the image below, is known as peristomal pyoderma gangrenosum. It is often mistaken for a wound infection or irritation from the appliance.

Peristomal pyoderma gangrenosum.

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Pyoderma gangrenosum may occur on the genitalia. This form, termed vulvar or penile pyoderma gangrenosum, must be differentiated from sexually transmitted diseases.^[16]One case report describes pyoderma gangrenosum of the scrotum in a patient with Crohn disease.^[17]

Extracutaneous neutrophilic disease may be evident upon ocular examination and has also been reported in the lungs, liver, and bones.^{[4, 14]}

Differential Diagnoses

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Ashchyan HJ, Butler DC, Nelson CA, Noe MH, Tsiaras WG, Lockwood SJ, et al. The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. JAMA Dermatol. 2018 Apr 1. 154 (4):409-413. [QxMD MEDLINE Link].
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Jackson JM. TNF- alpha inhibitors. Dermatol Ther. 2007 Jul-Aug. 20(4):251-64. [QxMD MEDLINE Link].
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Wilson DM, John GR, Callen JP. Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder:report of a patient with rheumatoid arthritis, pustular vasculitis,pyoderma gangrenosum, and Sweet''s syndrome with an excellent response tocyclosporine therapy. J Am Acad Dermatol. 1999 Feb. 40(2 Pt 2):331-4. [QxMD MEDLINE Link].
Daniels NH, Callen JP. Mycophenolate mofetil is an effective treatment for peristomal pyoderma gangrenosum. Arch Dermatol. 2004 Dec. 140(12):1427-9. [QxMD MEDLINE Link].
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August PJ, Wells GC. Pyoderma gangrenosum treated with azathioprine and prednisolone. Br J Dermatol. 1974. 91:80-2.
Burruss JB, Farmer ER, Callen JP. Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum. J Am Acad Dermatol. 1996 Nov. 35(5 Pt 1):720-4. [QxMD MEDLINE Link].
Campanati A, Brisigotti V, Ganzetti G, Molinelli E, Giuliodori K, Consales V, et al. Finally, recurrent pyoderma gangrenosum treated with Adalimumab: case report and review of the literature. J Eur Acad Dermatol Venereol. 2014 Sep 8. [QxMD MEDLINE Link].
Kaplan B, Trau H, Sofer E, Feinstein A, Schewach-Millet M. Treatment of pyoderma gangrenosum with clofazimine. Int J Dermatol. 1992 Aug. 31(8):591-3. [QxMD MEDLINE Link].
Johnson RB, Lazarus GS. Pulse therapy. Therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol. 1982 Feb. 118(2):76-84. [QxMD MEDLINE Link].
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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.