Cicatricial pemphigoid

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Cicatricial pemphigoid (benign mucosal pemphigoid, mucous membrane pemphigoid, MMP) is an immune-mediated subepithelial blistering disease that also tends to affects the elderly like pemphigoid. The mucosa only is involved, and while commonly gingiva and buccal mucosa, conjunctival scarring can lead to blindness. It thus tends to be more serious than bullous phemigoid due to scarring and complications such as blindness and strictures may be avoided by prompt aggressive treatment.

Aetiology

IgG or IgA, occasionally IgG4[1] against basement membrane antigens can be identified by immunofluorescence in most cases. The majority is to the 180 kD bullous pemphigoid antigen (BP180), with a quarter to the 230 kD bullous pemphigoid antigen (BPAg1)[2]. However other antigens such as β4 integrin or laminin 5 are involved in some cases, with the former being particularly common with ocular involvement[3].

Treatment

As for bullous pemphigoid but more likely to need cyclophosphamide or rituximab.

References

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