A brief review of pemphigus vulgaris

Abstract

Pemphigus vulgaris is an autoimmune disorder which presents with painful mucocutaneous blisters and erosions. On the skin, they are flaccid bullae or erosions, and on the mucosa, they present as erosions. This disease is rare but is devastating to those who have it; it also is related-perhaps genetically-to other autoimmune conditions. This is to say that a patient can develop pemphigus vulgaris if they have thyroiditis or diabetes mellitus. Biopsy is needed to obtain histopathological evidence of the breakdown of intercellular connections due to the autoimmune attack on components of desmosomes, which are responsible for intercellular integrity above the basement membrane. When these desmosomes are attacked, loss of connection ensues, and the cells break apart at these connections; this leads to fluid buildup, seen grossly as bullae. Treatment of the disease is difficult and sometimes unsafe. For decades, the mainstay of treatment has been glucocorticoids followed by other drugs. Unfortunately, these drugs are systemically absorbed, and the side effect profile can be unfavorable. In the past several years however, more innovative treatments have emerged that may help ease the cost and safety burden to patients. This review highlights the major points about pemphigus vulgaris, its pathophysiology, and its treatment.

Oral mucosal changes in pemphigus vulgaris and its treatment: A case report

“…[8] Intra-venous IgG can be administered in the maintenance phase with refractory disease un-responsive to other adjuvant drugs. [9] Pulsed therapy, such as intravenous dexamethasone–cyclophosphamide pulse therapy, has been used in the management of recalcitrant lesions. [10]…”

Register‐Studie zu Krankheitsentwicklung und Therapieansprechen bei Pemphigus

“…Das Hauptziel der Therapie beim Pemphigus ist eine Krankheitskontrolle, bei der Rezidive verhindert und die Nebenwirkungen von Glucocorticoiden und Immunsuppressiva reduziert werden [5]. In der Erstlinientherapie werden systemische Glucocorticoide häufig mit immunsuppressiven, steroidsparenden Adjuvantien kombiniert [3,6]. Bei therapierefraktärem Verlauf kommen Immunadsorption (IA) oder monoklonale anti-CD20-Antikörper (Rituximab) zum Einsatz.…”

“…Bei therapierefraktärem Verlauf kommen Immunadsorption (IA) oder monoklonale anti-CD20-Antikörper (Rituximab) zum Einsatz. Allerdings ist die chronische Verwendung systemischer Immunsuppressiva mit schwerwiegenden Langzeit-Nebenwirkungen assoziiert [6].…”

Disease progression and response to therapy in pemphigus based on a registry

“…The main objective of pemphigus therapy is to control the disease, to prevent relapses and to reduce side effects associated with the use of glucocorticoids and immunosuppressants [5]. As first-line therapy, systemic glucocorticoids are often combined with immunosuppressive steroid sparing adjuvants [3,6]. In the case of refractory therapy, immunoadsorption (IA) or anti-CD20 monoclonal antibody (rituximab), however, chronic use of systemic immunosuppressive therapies is associated with serious long-term side effects [6].…”