Skip to Main Content

AT-A-GLANCE

AT-A-GLANCE

  • Pyoderma gangrenosum (PG) is an uncommon, neutrophilic inflammatory skin condition, which classically presents as a painful nodule, plaque, or pustule that enlarges and breaks down to form a progressively enlarging ulcer with raised, undermined, violaceous borders and a surrounding zone of erythema. Healing PG lesions develop a cribriform appearance.

  • PG tends to occur and recur in areas of trauma because of the pathergic phenomenon, where trauma or irritation can induce flaring of PG.

  • PG most often occurs in association with systemic inflammation, and most reported cases have an associated underlying disease (such as inflammatory bowel disease, monoclonal gammopathy, hematologic disease, inflammatory arthritis, malignancy, hidradenitis suppurativa, etc); however, PG may precede these disorders. PG has also been reported in association with genetic mutations and syndromes, such as the pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome, or without an identifiable underlying disease.

  • PG most commonly presents as the classic ulcerative variant, but may also arise as bullous, pustular, or vegetative variants. Clinical features of different variants sometimes overlap in individual patients but usually one variant dominates the clinical picture.

  • There is no laboratory test or investigation that establishes the diagnosis of PG with certainty. The histopathologic findings are not diagnostic but can be supportive of the diagnosis of PG in the appropriate clinical setting and are essential to rule out alternative diagnoses.

  • Specified criteria (see “Diagnostic Algorithm” section) suggest the diagnosis of PG, but other conditions (particularly infection, vascular disease, and malignancy) must be excluded.

  • The mainstays of management are systemic immunosuppressive agents together with appropriate local and topical therapy.

  • Classic ulcerative PG is a chronic condition. Complete healing usually requires months of treatment; maintenance therapy is necessary in many and relapses are common. Significant morbidity and mortality are experienced by patients with ulcerative and bullous PG.

DEFINITIONS

Pyoderma gangrenosum (PG) is an uncommon, chronic, neutrophilic inflammatory skin condition, with the classic ulcerative variant typically presenting initially as a painful nodule, plaque, or pustule that enlarges and breaks down to form a progressively enlarging ulcer with raised, undermined, violaceous borders and a surrounding zone of erythema.1,2 The lesions are commonly very painful, develop a cribriform appearance with healing, and tend to occur and recur in areas of trauma in up to 50% of cases.1,2 PG is believed to occur in association with systemic inflammation, and most reported cases are associated with autoinflammatory or hematologic conditions, and typically respond to immune suppression.1,3-5 PG most commonly presents as the classic ulcerative variant, but may also present as bullous, pustular, or vegetative variants. Unless otherwise specified, when we refer to PG in this text, we are referring to the classic ulcerative variant.

EPIDEMIOLOGY

PG is estimated to affect up to 10 cases per million people per year, and represents up to 3% of chronic leg ulcer cases, but may occur ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.