Mucous membrane pemphigoid is a rare, chronic autoimmune disease characterized by subepidermal blistering and scarring, predominantly affecting mucous membranes. Ocular involvement frequently occurs and often represen...Mucous membrane pemphigoid is a rare, chronic autoimmune disease characterized by subepidermal blistering and scarring, predominantly affecting mucous membranes. Ocular involvement frequently occurs and often represents the only manifestation of the disease. We describe a 62- year- old woman with a bilateral 18- month duration of conjunctival hyperaemia, associated with erythema and oedema of the eyelids, lacking any typical ocular signs of mucous membrane pemphigoid such as subconjuctival fibrosis and scarring. Histology was not significant. Direct immunofluorescence of the conjunctiva showed IgG, IgA and complement deposition along the basement membrane zone. Immunoprecipitation analysis of affinity purified laminin- 5 revealed a band consistent with the β 3 chain of laminin- 5. This represents the first case of pure ocular mucous membrane pemphigoid associated with antilaminin- 5 antibodies.展开更多
文摘Mucous membrane pemphigoid is a rare, chronic autoimmune disease characterized by subepidermal blistering and scarring, predominantly affecting mucous membranes. Ocular involvement frequently occurs and often represents the only manifestation of the disease. We describe a 62- year- old woman with a bilateral 18- month duration of conjunctival hyperaemia, associated with erythema and oedema of the eyelids, lacking any typical ocular signs of mucous membrane pemphigoid such as subconjuctival fibrosis and scarring. Histology was not significant. Direct immunofluorescence of the conjunctiva showed IgG, IgA and complement deposition along the basement membrane zone. Immunoprecipitation analysis of affinity purified laminin- 5 revealed a band consistent with the β 3 chain of laminin- 5. This represents the first case of pure ocular mucous membrane pemphigoid associated with antilaminin- 5 antibodies.
