Pemphigus vulgaris: Therapeutic approach
Pemphigus vulgaris is a rare autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes. Loss of desmosome function results in loss of cohesion between keratinocytes in the epidermis, resulting in bullae and erosions affecting skin and mucous membranes.
The main objectives in pemphigus management are disease control, prevention of relapses, and avoidance of adverse events associated with the prolonged use of steroids and immunosuppressive agents. Due to its rarity, only a few prospective controlled therapeutic trials are available to guide management. Review of the therapeutic management will be discussed.
Systemic corticosteroid therapy is the starting point with treatment, generally used in combination with steroid-sparing immunosuppressants. First line immunosuppressant adjuvants include azathioprine and mychophenolate mofetil. Rituximab is a second line treatment that has been reported to be effective in recalcitrant pemphigus. Other second line treatments include infliximab, intravenous immunoglobulins, cyclophosphamide, immunoabsorption and plasmapheresis, methotrexate and dapsone. Other therapies reported include extracorporeal photophoresis and tumour necrosis factor inhibitors.
Theraputic challenges in management of pemphigus vulgaris will be illustrated with a case report of a fifty-five year old female with severe pemphigus vulgaris requiring multiple therapies.
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Dr. Eleni Anthony
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