A Case Series of Recalcitrant Pemphigus

Abstract

Pemphigus consists of a group of life threatening autoimmune bullous disorders characterised by flaccid blisters and erosions of the skin and mucosal membranes of oral, laryngeal, conjunctival, pharyngeal, anal, vaginal etc. Pemphigus vulgaris involves mucosa in 50-70% of the patients. Pemphigus is associated with other autoimmune disorders like myasthenia gravis and thymoma. Primary subsets of pemphigus include pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Pemphigus vulgaris constitutes nearly 70% of cases of pemphigus, with its antigens desmoglein 1 and 3, which are calcium dependent calmodulins, being the crucial targets for IgG1 and IgG4 classes of antibodies. If left untreated at an early stage, pemphigus vulgaris is associated with significant morbidity and mortality. The authors present a case series of five patients (34-year-old male, 68-year-old male, 52-year-old male, 74-year-old female and 65-year-old female) presenting recalcitrant pemphigus with focus on the various factors that affect the disease outcomes, morbidity and mortality.

Keywords

• deep vein thrombosis
• pemphigus vegetans
• resistant pemphigus
• septicaemia