Management of Pemphigus Vulgaris

Abstract

IntroductionPemphigus vulgaris (PV) is a chronic, autoimmune, vesiculobullous disease. As a result of the relative rarity of PV, published randomized controlled trials (RCTs) are limited, which makes it difficult to evaluate the efficacy of different treatment regimens in this disease. This also precludes conduct of a meta-analysis.MethodsEnglish-language publications describing treatment outcomes of patients with PV were identified by searches of electronic databases through May 2015, and additionally by review of the bibliography of these publications. A total of 89 papers, which included 21 case reports, 47 case series, 8 RCTs, and 13 observational studies, were identified. The findings from these publications, including information on disease course and prognosis, medications used, treatment responses, and side effects, are summarized in the tables and text of this review.ResultsPrior to availability of corticosteroid therapy, PV had a high fatality rate. Early publications from the 1970s reported high-dose, prolonged corticosteroid use and significant associated side effects. Later reports described use of corticosteroids along with steroid-sparing adjuvants, which allows a reduction in the total dose of corticosteroids and a reduction in observed mortality and morbidity. For the majority of patients in these reports, a long-term course on medications lasting about 5–10 years was observed; however, subgroups of patients requiring shorter courses or needing longer-term therapy have also been described. Early diagnosis of PV and early initiation of treatment were prognostic factors. In recent publications, commonly used initial regimens include corticosteroids in combination with mycophenolate or azathioprine; whereas, for patients with inadequate response to these regimens, adjuvants such as intravenous immunoglobulin (IVIg) or rituximab are used.ConclusionThe review findings emphasize the importance of early diagnosis, early initiation of treatment, and use of steroid-sparing adjuvants to allow a reduced total dose and duration on corticosteroids. Also highlighted is the need for more RCTs.

Comparison of the Expression Intensity of Estrogen Receptor Marker in Oral and Cutaneous Pemphigus Vulgaris

“…These autoantibodies cause loss of cell-cell adhesion in the epithelium, which results in suprabasilar intraepithelial vesicle formation. (13) As mentioned earlier, our results showed that the mean age of the patients with oral PV was 38.3±11.8 years with the female-to-male ratio equal to 1.5/1. These findings are consistent with the results of the study by Shamim et al (14) The average age of the patients in the mentioned study was 42.73 years with the femaleto-male ratio of 1.73/1.…”

“…In most cases, PV initially manifests as oral lesions. (13) This can explain the lower mean age of the patients with oral PV compared with that of the patients affected by cutaneous PV in the present study.…”

The imbalance of gut microbiota and its correlation with plasma inflammatory cytokines in pemphigus vulgaris patients

“…Pemphigus vulgaris (PV) is a life‐threatening autoimmune blistering disease clinically characterized by intra‐epidermal blisters and acantholysis resulting from the formation of IgG autoantibodies against desmoglein (Dsg)3, which impairs epidermal cell‐cell adhesion . Despite recent advances in the diagnosis and treatment of PV, the mortality rate of patients approaches 6%; moreover, several gaps persist in our understanding of the pathogenesis of this disease .…”

The effect of l‐carnitine supplementation on serum levels of omentin‐1, visfatin and SFRP5 and glycemic indices in patients with pemphigus vulgaris: A randomized, double‐blind, placebo‐controlled clinical trial

“…Pemphigus vulgaris (PV) is a chronic, blistering, autoimmune disorder that affects the skin and mucus membranes and could be fatal if left untreated (Kridin, Shihade, & Zelber‐Sagi, ; Samadi, Gorouhi, Davari, & Firooz, ). Although early diagnosis and management of PV is important for the reduction of mortality rate, there is no curative treatment for PV patients so far (Cholera & Chainani‐Wu, ). Several newly available drugs including corticosteroids, intravenous immunoglobulins, and immunosuppressive agents improved prognosis and survival among PV patients (Tavakolpour, ).…”

“…Pemphigus vulgaris (PV) is a chronic, blistering, autoimmune disorder that affects the skin and mucus membranes and could be fatal if left untreated (Kridin, Shihade, & Zelber-Sagi, 2018;Samadi, Gorouhi, Davari, & Firooz, 2007). Although early diagnosis and management of PV is important for the reduction of mortality rate, there is no curative treatment for PV patients so far (Cholera & Chainani-Wu, 2016).…”