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We report a case of successful combined therapy for rapidly progressive pyoderma gangrenosum (PG). A 78 year old woman was admitted to the department of rheumatology with large right leg skin ulcers affecting the anterior and mediolateral lower shin region (fig 1A). The lesions had developed from small pustules to a focus of large painful skin damage during the past month. Her past history was free of any skin disease. Arterial hypertension, depression, and osteoporosis were controlled with permanent treatment. Her laboratory findings were unremarkable, including negative hepatitis B surface antigen and hepatitis C virus and normal liver function.
A marginal biopsy was performed, which highlighted a massive neutrophil infiltrate invading subcutaneous fat with necrotic debris associated with PG, with no evidence of vasculitis, malignancy, or infection. The patient had had seropositive rheumatoid arthritis for 32 years and her joint disease was controlled with long term treatment with azathioprine 150 mg/day. Despite the large rapidly progressive skin lesion and threat of leg amputation we decided to stop azathioprine and to begin combined therapy. This treatment included intravenous monthly cyclophosphamide pulse therapy 1000 mg (20 mg/kg), oral cyclosporin A 100 mg/day (2 mg/kg), a moderate dose of prednisone 30 mg/day (0.6 mg/kg) with tapering to 20 mg/day after 2 months, and a local treatment. To achieve a clean wound, she underwent one session of maggot debridement treatment (MDT). After confirming a normal ankle brachial index by duplex we started to apply two layer pressure dressings with occasional granulation tissue promoters. After 3 months of the treatment the wound healed completely (fig 1B). Three additional monthly pulses of cyclophosphamide were given. The patient’s present treatment comprises cyclosporin A 2 mg/kg and prednisone 10 mg/day. She is well, has normal daily living, and no active arthritis.
PG is a non-infectious neutrophilic dermatosis that usually starts with sterile pustules, which rapidly progress to painful ulcer with undermined violaceous borders. In 17–74% cases, PG is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or haematological disease, or malignancy. Diagnosis of PG is based on a history of an underlying disease, typical clinical presentation, and histopathology, and exclusion of other diseases that would lead to a similar appearance. Despite recent advances in treatment, the prognosis of PG remains unpredictable.1 The optimal treatment of PG includes a combination of local wound care and systemic treatment. Often, it is difficult to control aggressive cases, necessitating administration of a combination of systemic treatments.2 We followed this way and administered first line treatment of high dose corticosteroids and cyclosporin A3 along with monthly pulses of cyclophosphamide.4,5 MDT was first introduced in America in 1931. Sterile maggots of the green bottle fly Lucilia (Phacnicia) scricata are used for MDT. Up to 1000 maggots are introduced into the wound and left for 1–3 days. One of the major advantages of MDT is that the maggots separate the necrotic tissue from the living tissue. In 80–95% of cases a complete or significant debridement of the wound is achieved. An immediate amputation can be prevented as a result of MDT.6 In another study 21 ambulatory patients with non-healing wounds were treated with MDT.7 Of the eight patients who were advised to undergo amputation or major surgical debridement, only three required surgical resection (amputation) after MDT. Eleven healed without any additional surgical procedures.7 It is a simple, efficient, well tolerated, and cost effective tool for the treatment of wounds and ulcers which do not response to conventional treatment.6 We used it as first line treatment together with systemic treatment and achieved a rapid and complete effect.