Dermatologic Emergencies

dermatology-emergencieskapak-1-0-25

Pemphigus Vulgaris

Yasin Burak YILMAZ
Ankara Bilkent City Hospital, Clinic of Emergency Medicine, Ankara, Türkiye

Yılmaz YB. Pemphigus vulgaris. In: Oğuztürk H, Görmeli Kurt N, eds. Dermatologic Emergencies. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.36-40.

ABSTRACT

Pemphigus is a group of potentially life-threatening autoimmune bullous diseases characterized by acantholysis, which results in intraepithelial blister formation in the mucous membranes and skin. The most common form is pemphigus vulgaris, a rare disease whose incidence varies by geography and ethnicity. It is most frequently observed among Ashkenazi Jews, as well as populations in India, Southeast Europe, and the Middle East. The pathogenesis of pemphigus involves autoantibodies, primarily IgG, against desmogleins (components of the desmosomes) that cause the loss of cell adhesion between keratinocytes. This leads to acantholysis and blister formation. In pemphigus vulgaris, IgG autoantibodies target desmoglein 3 (primarily mucosal involvement) or both desmoglein 1 and 3 (mucocutaneous involvement). Nearly all patients with pemphigus vulgaris develop mucosal involvement, with the oral cavity being the most common site. Other mucosal regions like the conjunctiva, esophagus, and genital areas can also be affected. Painful flaccid bullae and erosions develop on the skin, and Nikolsky’s sign (induced blistering by applying pressure to the skin) is often positive. Pruritus is typically absent. Diagnosis is based on clinical, histological, immunopathological, and serological findings. Pemphigus vulgaris must be distinguished from other autoimmune bullous diseases, such as bullous pemphigoid, linear IgA dermatosis, and epidermolysis bullosa acquisita, which typically present with tense bullae rather than the flaccid ones seen in pemphigus. Treatment centers around pain control and local wound care, alongside systemic therapies. Systemic glucocorticoids are the cornerstone, often combined with rituximab for rapid disease control. Adjuvant therapies, such as mycophenolate mofetil or azathioprine, are often used alongside glucocorticoids to mitigate side effects and reduce steroid dependency. Complications from treatment can be significant. Rituximab carries risks such as infusion reactions and rare instances of progressive multifocal leukoencephalopathy. Long-term glucocorticoid use can lead to hypertension, osteoporosis, diabetes, and increased susceptibility to infections, while adjuvant drugs also pose risks of myelosuppression, gastrointestinal disorders, and infections.

Keywords: Pemphigus; rituximab; glucocorticoids; pemphigus vulgaris; familial

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