Long-term maintenance therapy with Cyclosporine A in adults with generalized pustular psoriasis

摘要

Generalized pustular psoriasis (GPP) is a rare and serious immune-mediated skin disorder that is characterized by a widespread eruption of sterile and subcorneal pustules. In the present study we investigated the efficacy of Cyclosporine A (Cy A) in treatment of 9 adults with drug-refractory GPP viz. topical Corticosteroids, retinoids, methotrexate and narrow-band ultraviolet light exposure (UVB). Initially; they were resuscitated as burn patients. Cy A was administered on day 1 at a dose of 100 mg twice daily either in the form of syrup or Neoral capsules. In most patients, skin lesions had healed by 6 weeks and the dose of Cy A was reduced to minimum to prevent further recurrence. Seven patients had required 50 mg twice daily and 2 were controlled with 50 mg am and 25 mg pm. On follow up, there was no serious relapse, liver and kidney disease. Minor complications included; hirsutism and dark skin (n: 5) and gingival hyperplasia (n: 2). Trial to replace Cy A with Tacrolimus (Prograf) failed to maintain remission. In conclusion; Cy A is a safe and effective treatment for GPP.