Table 1.
Summary of clinical characteristics, etiopathogenesis and paraclinical findings in different autoimmune bullous diseases.
| Disease | Ocular Findings | Systemic Findings | Etiopathogenesis | Histology/laboratory |
|---|---|---|---|---|
| MMP | Chronic cicatrizing conjunctivitis, subconjunctival bullae, conjunctival ulceration, pseudomembrane formation, fornix shortening, symblepharon, trichiasis, dry eye, corneal epithelial defect, ulceration and vascularization, ocular surface keratinization | Oral (100%), pharynx (43%), nasal mucosa (38%), larynx (30%), genital mucosa (20-35%), rectum (11%), esophagus (7%), skin (10-43%) | Type-2 hypersensitivity reaction, autoantibodies against BMZ antigens (BP180, BPAG 2) | Subepithelial bullae, Linear immunoglobulin and complement deposition along BMZ detected by DFA or immunoperoxidase assay, circulating anti-BM autoantibodies |
| PV | Non-cicatrizing conjunctivitis, conjunctival erosions, lid margin erosions (especially medial aspect of the lower eyelid) | Skin, oral mucosa, larynx, esophagus, anus, cervix, vulva | Autoantibodies against desmosomal antigens desmoglein 1 and 3 | Suprabasal (intra-epithelial) blister, intercellular immunoglobulin and complement deposits detected by DFA, anti-desmoglein 1 and 3 autoantibodies detected by ELISA |
| PNP | Similar to MMP. Bilateral cicatrizing conjunctivitis, subconjunctival bullae, conjunctival ulceration, pseudomembrane formation, fornix shortening, symblepharon, dry eye, corneal epithelial defect, ulceration and melting | Oral mucosa (severe intractable stomatitis), nasopharynx, oropharynx, nasal septum, hypopharynx, larynx, tracheobronchial mucosa, esophagus, epiglottis, skin (latent) | Autoantibodies with cross-reactivitiy against epidermal (desmosomal) antigens | Suprabasal acantholysis, necrosis of epidermal keratinocytes, intercellular and/or BMZ deposition of IgG and complement |
| LAD | May resemble MMP, may be asymptomatic. Conjunctival scarring, subconjunctival fibrosis, trichiasis, entropion, inferior fornix shortening and symblepharon with secondary corneal scarring | Annular tense bullae in perioral region, perineum, and extremities | Autoantibodies against LAD-1/collagen type VII Drug-related: vancomycin, captopril, amiodarone, phenytoin. Secondary to preceding illness, malignancy, or other autoimmune disorder | Subepidermal bullae, linear deposition of IgA and complement C3 along the BMZ detected by DFA, circulating anti-BMZ autoantibodies |
| EBA | Chronic conjunctivitis, symblepharon, keratitis, subepithelial corneal vesiculation, perforation, scarring | Milia and scarring at sites of trauma (e.g. elbows, knees, buttock, dorsa of feet and hands) and nasal, pharyngeal, esophageal, and genital mucosa | IgG autoantibodies against collagen type VII, disruption of lamina densa of the EBM | Subepidermal blister with intact epidermis, linear thick band IgG deposit along BMZ detected by DFA, circulating IgG autoantibodies against type VII collagen using ELISA or IFA |
BMZ, basement membrane zone; BM, basement membrane; EBM, epithelial basement membrane; MMP, mucus membrane pemphigoid; PV, pemphigus vulgaris; PNP, paraneoplastic pemphigus; LAD, linear IgA disease; EBA, epidermolysis bullosa acquisita; DFA, direct immunofluorescent assay; IFA, indirect immunofluorescent assay.