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Pyoderma Gangrenosum

Treatment

First-line therapy: The first-line therapy for pyoderma gangrenosum is comprised of two key elements: controlling inflammation and optimizing wound care. Inflammatory control may require systemic immunosuppression and/or the use of multiple therapeutic agents in combination.

First steps

  • Systemic corticosteroids are the initial treatment of choice for patients with established or multiple ulcerations, rapidly expanding lesions. Initial therapy is 1 mg/kg or higher per day. After a response is seen, gradually taper the dose. If no response is seen in a week, a second immunosuppressive agent should be added.
  • Local therapy is very effective for early, superficial, and those lesions that are not rapidly progressive. Consider these options in addition to optimal wound care:
  • PG lesions are easily inflamed; surgical debridement or grafting should generally be avoided, as it will lead to exacerbation of the lesion.

Ancillary steps

  • Vapor-permeable, moisture-retentive, hydrophilic membranes help control pain and may hasten re-epithelialization. Dressings can remain in place for 3-7 days. Dressings on exudative ulcers should be changed frequently to avoid maceration; alternatively, ulcers should be covered by a non-adherent dressing with absorptive features (such as foam or alginate). Removal must be very gentle to avoid pathergy (appearance of disease in sites of trauma).
  • Work-up for an associated disease is mandatory, since control of PG often can be achieved with therapy of the underlying disease process. Inflammatory bowel disease (i.e., both ulcerative colitis and regional enteritis [Crohn's]) is the most common associated illness. Other systemic disorders associated with PG include rheumatologic disease, malignancy (especially hematologic), infection, immunodeficiency, and medications. The diagnostic work-up, especially for an underlying malignancy, is also required as treatment of patients with PG may necessitate systemic immunosuppression, which may be relatively contraindicated in the setting of malignancy.
  • Healed lesions of PG are extremely susceptible to trauma, which can reactivate previously quiescent lesions. Emollients should be employed routinely, and both elevation and support stockings should be prescribed to minimize venous pooling that can lead to leg ulcers.

Subsequent steps

  • If the response to systemic steroids is inadequate and the process is extremely rapid, treat with cyclosporine A at an initial dose of 5-10 mg/kg/day (in addition to systemic steroids) with close monitoring for side effects. Response should be dramatic and will permit rapid reduction of systemic steroids (as long as cyclosporine complications are not encountered).
  • In superficial lesions and those with limited progression, addition of minocycline, dapsone, colchicine, clofazamine, thalidomide may improve the PG lesion.
  • In refractory cases, pulsed systemic steroids, or addition of mycophenolate mofetil, anti-tumor necrosis factor blockade, or azathioprine to systemic steroids may halt the progression of disease.

Pitfalls

  • Pyoderma gangrenosum-like lesions occur in a host of other diseases, including carcinomas, mycobacterial and other infections, halogenoderma, gummatous syphilis, Wegener’s granulomatosis, polyarteritis nodosa, antiphospholipid antibody syndrome, cholesterol emboli, and can be mimicked by factitial ulcers. At the institution of therapy, strong consideration should be given to performing an incisional biopsy for routine histology and cultures, and performing appropriate laboratory tests to exclude the above conditions.
  • Pathergy occurs in many patients with PG, thus all but the most gentle debridement or other manipulations should be avoided.

When to refer to a dermatologist

  • When the diagnosis of pyoderma gangrenosum is not clear.
  • For biopsy of the ulcerating lesion to exclude an infectious or inflammatory condition mimicking PG.
  • For co-management of systemic immunosuppression and wound healing.