Synonym
Cicatricial pemphigoid
Definition
Mucous membrane pemphigoid (MMP), previously known as “cicatricial pemphigoid,” (Chan et al. 2002) is a chronic, autoimmune, subepithelial blistering disorder characterized by involvement of predominantly mucosal surfaces and a tendency for scarring.
HLA type DQB1*0301(DQ7) is common amongst patients with both MMP and classic bullous pemphigoid.
Clinical
A chronic and progressive disease, MMP may be associated with substantive morbidity due to the tendency for scarring and fibrosis. When present on the conjunctivae, these effects may lead to blindness.
The histopathologic presentation of MMP is similar to that observed in classic bullous pemphigoid but with fewer eosinophils and more plasma cells being present in the lymphohistiocytic infiltrate that surrounds the subepidermal blisters. Mast cells and other mononuclear leukocytes are also found. Later stages also show granulation tissue in the submucosa and the characteristic fibrosis in the...
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References
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Sofen, B.D., Soter, N.A. (2014). Mucous Membrane Pemphigoid. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_254
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DOI: https://doi.org/10.1007/978-0-387-84828-0_254
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