Open AccessA Case Series of Recalcitrant Pemphigus
Author(s) -
Rajkumar Kannan,
Samuel Jayaraj Daniel,
. Poornima,
Ramesh Aravamuthan
Publication year - 2022
Publication title -
journal of clinical and diagnostic research
Language(s) - English
Resource type - Journals
eISSN - 2249-782X
pISSN - 0973-709X
DOI - 10.7860/jcdr/2022/53400.16084
Subject(s) - pemphigus vulgaris , pemphigus foliaceus , pemphigus , medicine , paraneoplastic pemphigus , desmoglein 3 , dermatology , desmoglein , autoimmune disease , acantholysis , desmoglein 1 , immunology , autoantibody , antibody
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 recalcitrant pemphigus with focus on the various factors that affect the disease outcomes, morbidity and mortality