Rapid control of therapy-refractory pemphigus vulgaris by treatment with the tumour necrosis factor-alpha inhibitor infliximab

Conflicts of interest: none declared. Sir, We report on a 43‐year‐old male who had an 8‐year history of severe, therapy‐refractory pemphigus vulgaris (PV) with a history of pulmonary embolism, arterial hypertension, steroid‐induced osteoporosis and diabetes mellitus who presented with disseminated cutaneous blisters and extensive erosions of the oral mucosa. Immunosuppressive therapy including glucocorticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil and leflunomide did not lead to long‐lasting disease remissions. When the clinical status of our patient further deteriorated on treatment with leflunomide (20 mg day−1) and prednisone (20 mg day−1), we had to search for therapeutic alternatives. The recent finding that tumour necrosis factor (TNF)‐α was directly involved in the process of intraepidermal loss of adhesion in animal models of PV prompted us to initiate a therapeutic trial with the TNF‐α blocker infliximab. The patient was given three doses of infliximab (5 mg kg −1day−1 intravenously) over 6 weeks at weeks 0, 2 and 6. Leflunomide and prednisone were continued during the infliximab regimen as adjuvant treatment and with the aim of preventing formation of anti‐infliximab antibodies because a subgroup of patients treated with infliximab develop neutralizing antibodies. Therefore infliximab has been routinely prescribed with methotrexate for rheumatoid arthritis to prevent the development of these antichimeric antibodies.1 Within 48 h of the first dose of infliximab, blister formation ceased and within the first 7 days, cutaneous blisters and mucosal erosions began to heal (Fig. 1). During this time the patient received topical treatment with inert cooling emollients (unguentum leniens) and lesional povidone–iodine. Infliximab infusions were discontinued 8 weeks after initiation when the patient developed varicella zoster of the left S1/2 dermatome which was treated successfully with intravenous aciclovir. Of note, the patient's clinical status remained stable with few cutaneous erosions on the previously insufficient therapy with leflunomide (20 mg day−1) and prednisone (20 mg day−1). After a relapse 4 months later the disease was controlled by several cycles of immunoadsorption therapy in combination with mycophenolate mofetil (3 g day−1).