Pyoderma Gangrenosum Clinical Presentation

Updated: Sep 05, 2018
  • Author: J Mark Jackson, MD; Chief Editor: William D James, MD  more...
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Presentation

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. 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 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]

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

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. Peristomal pyoderma gangrenosum.

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]

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