Severe Multi-Resistant Pemphigus vulgaris: prolonged remission with a single cycle of Rituximab

Abstract

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120mg, soon reduced to 20mg.

An assessment of treatment history and its association with clinical outcomes and relapse in 155 pemphigus patients with response to a single cycle of rituximab

“…In total, we recorded data of 155 pemphigus patients, 124 of whom had PV while 31 had PF patients with ages ranging from 4 to 86 years . There was no association between disease type and clinical outcomes, with 80% of both PV and PF patients achieving complete response ( P = 0.95).…”

Rituximab therapy improves recalcitrant Pemphigus vulgaris

“…Additionally, it has been shown that patients treated with rituximab earlier in the disease course may have better outcomes, enduring complete remission with no or minimal systemic therapy for 19 months (Lunardon et al, 2012[ 21 ]). Other studies also indicated that adjuvant rituximab therapy may hold promises in long-lasting treatment of severe recalcitrant PV with no or slight adverse drug-related events (Baum et al, 2013[ 4 ]; Behzad et al, 2012[ 5 ]; Cho et al, 2014[ 7 ]; Corral et al, 2013[ 9 ]; Craythorne et al, 2011[ 10 ]; El Tal et al, 2006[ 11 ]; Eming et al, 2008[ 12 ]).…”

Kaposi Varicelliform Eruption in a Patient with Pemphigus Vulgaris: A Case Report and Review of the Literature

“…Pemphigus vulgaris (PV) is an autoimmune intraepidermal blistering disorder of the skin and mucous membrane caused due to damage to intercellular adhesion structures, i.e., desmogleins resulting in acantholysis [ 1 ]. Kaposi varicelliform eruption (KVE) is a rare and potentially fatal viral infection typically due to herpes simplex virus (HSV) 1 or 2, rarely by coxsackie A16 virus and vaccinia virus over a pre-existing dermatosis [ 2 ].…”