CASE HISTORY – February 2014

Pemphigus vulgaris – an interdisciplinary disease

Authors: Kris Lutter, Ott Kiens, Eevi Pärsik

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Pemphigus vulgaris is a rare severe autoimmune blistering skin disease with painful skin lesions. It is caused by antibodies directed against desmoglein 1 and 3. This results in loss of cohesion between keratinocytes in the epidermis and is therefore characterized by extensive blisters and mucocutaneous erosions. Pemphigus can occur at any age but it often strikes middleaged or older people. The gold standard for the diagnosis is punch biopsy which is confirmed by direct immunofluorescence staining or by indirect immunof luorescence test.

Without treatment it is a potentially life-threatening disease with a mortality rate of approximately 5-15%. According to the 2003 guidelines published in the British Medical Journal, before corticosteroids were introduced in the 1950s, death rate for this disease averaged 75 percent. The aim of pharmacologic therapy for pemphigus vulgaris is to reduce inflammatory response and autoantibody production. Treatment with both corticosteroids and other immunosupressive drugs like IV Ig, Rituximab, Mycophenolate mofetil, Methotrexate, Azathioprine, and Cyclophosphamide enables a delay of adverse events. Early diagnosis with appropriate initial and maintenance drug dosage is the key to better prognosis and lower mortality rates. Also regular follow-up with proper modification of treatment is neccessary for long-term remission.