Life-threatening course of pemphigus vulgaris complicated by sepsis caused by azathioprine-induced bone marrow suppression, successfully managed with combination therapy

Abstract

Severe forms of pemphigus vulgaris (PV) that are resistant to standard treatment present a life-threatening disease with a mortality of 5-10%. The treatment is usually individualized. The most popular procedures used today include intravenous applications of immunoglobulins and rituximab. Currently the common use of pulse corticosteroids, often in first-line treatment, is being neglected. This particular case documented the severity of the disease and also the need for combined and comprehensive care, in which corticosteroid pulse therapy still plays an important role.

Pathophysiology of autoimmune bullous diseases: Nature versus nurture

“…Prior to the discovery of an effective therapy, PV was fatal in approximately 70% of cases as a result of fluid/electrolyte-associated abnormalities and infection. [13]…”

Dermatolojide Rituksimab Kullanımı

“…B hücre populasyonu ise düşük doz alan grupta daha erken oluşmuştur. Sonuç olarak yazarlar yüksek doz rituksimab tedavisinin bazı klinik ve laboratuvar parametrelerde daha etkin düzelme sağlayabileceği sonucuna varmışlardır (58) (61). Shimanovich ve ark ise 5 şiddetli pemfiguslu olguyu protein A immunoadsorbsiyon, rituksimab ve İVİG kombinasyonuyla başarılı şekilde tedavi edebilmiştir (98).…”