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Index: Ostomy Wound Manage. 2008;54(11):32-36.

Pyoderma gangrenosum (PD) is a rare, chronic, relapsing, ulcerative, neutrophilic cutaneous disease and may be difficult to recognize. It is not uncommon for PD to be mistakenly diagnosed as vascular occlusive or venous disease, vasculitis, cancer, infection, exogenous tissue injury, or other inflammatory disorders. A 55-year-old woman with a 5-year history of a very painful and enlarging ulcer presented at the authors’ clinic. Previously, based on an original diagnosis of venous ulcer, the wound had been surgically debrided and managed with saline-soaked gauze and compression therapy. After the authors secured a complete history (which included rheumatoid arthritis) and assessment, PD was suspected. A biopsy was performed for histological confirmation. Pyoderma gangrenosum treatment, including oral corticosteroids and topical 0.01% tacrolimus twice daily covered with nonadhesive gauze and compression wrapping, was started. After 4 weeks, the wound had improved noticeably and pain medications to manage wound pain were discontinued. The wound was completely healed after 4 months. The presence or absence of PD must be ascertained in all patients who present with a history of painful lower leg ulcers and PD risk factors, such as rheumatoid arthritis.

KEYWORDS: case study, pyoderma gangrenosum, skin ulcer, diagnosis

     Pyoderma gangrenosum (PG) is a rare, chronic, ulcerative, cutaneous disease with recurrent relapses, one of the neutrophilic diseases that affect the skin. It was first described by Brocq in 1916 and later named by Brunsting in 1930.1 The cause of PG remains unknown but results of a study¹ of biopsy specimens from patients with PG suggest that neutrophil chemotaxis is altered. Pyoderma gangrenosum has a worldwide distribution and can appear at any age in either gender but is most frequently diagnosed in women between 20 and 50 years old.²

     In 50% of all cases, PG is associated with systemic disease, most commonly inflammatory bowel disease(30% to 60%).² A monoclonal gammopathy is present in approximately 10% of patients with PG; the frequency of malignant disease in patients with PG is uncertain, but 7% is a reasonable estimate.³ Pyoderma gangrenosum also has been described in patients with chronic active hepatitis, myeloma, polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria, Takayasu’s arteritis, primary biliary cirrhosis, systemic lupus erythematosus, and HIV infection.^2,4-12 One case of pyoderma gangrenosum associated with retinoid therapy has been reported.¹³

     The purpose of this review and case study is to describe and illustrate clinical diagnostic and treatment strategies.

Type and Course

     Clinically, PG may be designated as classic, atypical, and peristomal. The classic form is characterized by well-demarcated ulcers in the legs that begin as painful pustules or vesicles that become necrotic with overhanging borders. The pustules develop further necrosis and tend to grow and deepen over time. The base of the ulcer is suppurative and the borders undermined and violet. Loss of tissue may expose the underlying tendons. These ulcers may be unique or multiple and are located mainly on the legs. They often worsen after debridement (pathergy).

     Atypical PG is usually found on the face, hands, or arms and is characterized by superficial ulcers with a blue-gray, bullous border. Peristomal PG occurs around stomas in patients with inflammatory bowel disease.¹⁴

     The course of PG can be acute (uniphasic), relapsing, or chronic.