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Ocular surface involvement in pemphigus vulgaris: An interdisciplinary review

The Ocular Surface(2020)

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Abstract
•PV is a humoral autoimmune blistering disease.•Auto-reactive IgG4 bind Dsg3 and affect binding and intracellular signaling.•Mucous membrane PV targets desmosomal Dsg3.•Mucous membrane pemphigoid targets hemi-desmosomal/lamina Lucinda antigens.•DSG3 is expressed on the entire ocular surface.•95% of PV patients possess MHC DRB1*0402 or DQQB1*0503.•Ocular findings are seen in up to 16% of PV patients.•Conjunctivitis is the most common eye finding of PV followed by dry eyes, scarring is rare.•Diagnosis of PV is made by tissue biopsy and direct immunofluorescence against IgG.•The anti-CD20 drugs are the most promising steroid-sparing agents in PV.
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Key words
Ocular,Conjunctivitis,Pemphigus vulgaris,Desmoglein 3,Acantholysis,DRB1*0402,DQB1*0503,ST18,p38MAPK,Muscarinic acetylcholine receptors
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