British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian

Disclosures

The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Background

PV is an acquired autoimmune disease in which immunoglobulin G (IgG) antibodies target desmosomal proteins to produce intraepithelial, mucocutaneous blistering. Desmoglein 3 is the major antigen, but 50–60% of patients have additional antibodies to desmoglein 1, the antigen targeted in pemphigus foliaceus (PF).[3–5] Although the pathogenesis of PV is complex, involving multiple pathways,[6] the underlying antibody profile is a major determinant of the clinical phenotype of PV.[5,7,8]

The average mortality of PV was 75% before the introduction of corticosteroids in the early 1950s.[9] This figure may be an underestimate due to the lack of diagnostic criteria, and inclusion of all subtypes of pemphigus and of other blistering disorders such as bullous pemphigoid, which have a better prognosis. However, not all cases of PV have such a dismal prognosis. Studies differentiating the clinical phenotypes have shown a lower mortality in patients with predominantly mucosal PV (1–17%) compared with those with mucocutaneous PV (8–42%).[10–12] Mucocutaneous PV tends to be a more severe disease, proving slower to respond to treatment and less likely to achieve remission off-treatment than purely mucosal PV.[13]

Clinical Presentation

The diagnosis of PV should be suspected in any patient with mucocutaneous erosions or blisters. The oral mucosa is the first site of involvement in the majority of cases, and PV may remain confined to the mucosal surfaces or extend to involve the skin (average lag period of 4 months).[14–16] Diagnostic delay is very common when PV is confined to the oral mucosa.[17] A minority of patients will present with cutaneous erosions, but oral erosions will, eventually, occur in most cases. PV presents across a wide age range with peak frequency in the third to sixth decades.

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