Mucous membrane pemphigoid

Mucous membrane pemphigoid is a heterogeneous group of chronic, autoimmune, subepithelial, blistering diseases which predominantly involve the mucous membranes (mainly oral and ocular) and occasionally the skin. It is chronic with an autoimmune aetiology, rather rare, and more common in patients between 50 and 70 years of age, especially females. Its pathology involves the production of autoantibodies which are directed towards various protein components of the haemidesmosomes, a connecting structure between the epithelium and the underlying connective tissue, whose destruction causes the formation of blisters under the epithelium.
It appears clinically in the oral cavity as a blister formation, surrounded by erythematous rings. This formation on rupturing (with a loss of the upper part of the blister) results in ulcerative lesions which are subject to scar formation. On occasion the appearance of the mucosa appears normal or slightly erythematous but, if subject to slight mechanical stimulation, an epithelial dissociation can occur with the formation of blisters (Nicolsky’s sign), whose outermost layer can sometimes be lost.
The lesions are very painful and associated with alterations of stomatological functions (phonotary, deglutary and masticaty). A frequent oral clinical variant of mucous membrane pemphigoid, involving the gingival tissue, is desquamative gingivitis; the latter is often erroneously diagnosed as gingivitis, although in the absence of plaques.
The diagnosis of pemphigoid is performed by biopsy (which renders the intra-epithelial blisters visible) and direct immunofluorescence (as evidence of IgG and C3 linear deposits in the intercellular spaces with a typical lattice morphology).
Research using autoantibody serum is also very useful (indirect immunofluorescence) in understanding this disease. On account of its tendency to affect various parts of the body, mucous membrane pemphigoid can have very serious consequences on the life of the patient who, when in an acute stage, must be treated by a multi-specialist team offering suitable systemic treatment (intravenously or orally ) with an immunosuppressant action.