Ocular 'nonscarring' mucous membrane pemphigoid associated with antilaminin-5 antibodies

Abstract

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 sub-conjuctival 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 beta3 chain of laminin-5. This represents the first case of pure ocular mucous membrane pemphigoid associated with anti-laminin-5 antibodies.

A Case of Linear IgA/IgG Bullous Dermatosis with Anti-laminin-332 Autoantibodies

“…Our patient without mucosal involvement should be differentiated from IgA type anti-laminin-332 MMP, only 3 cases of which have been reported thus far (9)(10)(11). Two cases had severe mucous membrane involvement and were refractory to treatments (9, 10), while another case had only ocular lesion (11).…”

“…Two cases had severe mucous membrane involvement and were refractory to treatments (9, 10), while another case had only ocular lesion (11).…”

Differences in basement membrane zone components of normal conjunctiva, conjunctiva in glaucoma and normal skin

“…2005). In contrary to generalized MMP, patients with typical ocular MMP rarely develop autoantibodies to laminin 5 (Coronella et al. 2005).…”

“…The a3 subunit of epilegrin is considered the main target antigen in epilegrin-associated MMP-like disease (Fujimoto et al 1990;Kirtschig et al 1995Kirtschig et al , 1998Dainichi et al 2005). In contrary to generalized MMP, patients with typical ocular MMP rarely develop autoantibodies to laminin 5 (Coronella et al 2005). This clinical observation may be explained by the virtual absence of b3 and c2 subchains of laminin in the normal conjunctiva.…”

Ocular cicatricial pemphigoid

“…Coronella et al [23] describe the first reported case of pure OCP with circulating antibodies to the b3 chain of laminin 5 (detected by immunoblot and immunoprecipitation analysis) but no other recognized MMP autoantibodies. This patient was unusual in that there was ocular surface inflammation but no clinical evidence of conjunctival scarring.…”