Pemphigus

Pemphigus encompasses a group of potentially life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin.

Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa.

Pemphigus foliaceus (PF) is confined to the skin. Paraneoplastic pemphigus (PNP) is rare; it can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and esophagus.

Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both.

Direct immunofluorescence staining of biopsied skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis.

Treatment aims to reduce or eliminate pathologic autoantibodies and consists of long-term immunosuppressive therapy.

Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are three broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP).

Underlying intraepithelial blister formation is caused by IgG autoantibodies against desmoglein 3 (Dsg3) and/or desmoglein 1 (Dsg1) on the cell surface of epidermal keratinocytes.[1]Joly P, Horvath B, Patsatsi Α, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020 Sep;34(9):1900-13. https://onlinelibrary.wiley.com/doi/10.1111/jdv.16752 http://www.ncbi.nlm.nih.gov/pubmed/32830877?tool=bestpractice.com

Pemphigus is associated with pain and impaired sleep. Swallowing difficulties are present if there is esophageal involvement. It is associated with increased rates of depression.[2]Hsu YM, Fang HY, Lin CL, et al. The risk of depression in patients with pemphigus: a nationwide cohort study in Taiwan. Int J Environ Res Public Health. 2020 Mar 17;17(6):1983. https://www.mdpi.com/1660-4601/17/6/1983/htm http://www.ncbi.nlm.nih.gov/pubmed/32192212?tool=bestpractice.com