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.
IgG or IgA, occasionally IgG4 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). However other antigens such as β4 integrin or laminin 5 are involved in some cases, with the former being particularly common with ocular involvement.
- ↑ Suresh L, Kumar V. Significance of IgG4 in the diagnosis of mucous membrane pemphigoid. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 2007 Sep; 104(3):359-62.(Link to article – subscription may be required.)
- ↑ Carrozzo M, Cozzani E, Broccoletti R, Carbone M, Pentenero M, Arduino P, Parodi A, Gandolfo S. Analysis of antigens targeted by circulating IgG and IgA antibodies in patients with mucous membrane pemphigoid predominantly affecting the oral cavity. Journal of periodontology. 2004 Oct; 75(10):1302-8.
- ↑ Oyama N, Setterfield JF, Powell AM, Sakuma-Oyama Y, Albert S, Bhogal BS, Vaughan RW, Kaneko F, Challacombe SJ, Black MM. Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity. The British journal of dermatology. 2006 Jan; 154(1):90-8.(Link to article – subscription may be required.)