LABD is a rare subepidermal blistering disease characterised by subepidermal blister formation and linear deposition of immunoglobulin A basement membrane antibodies. The target antigen is a key component of the dermoepidermal adhesion complex [21]. The mucous membranes including the eye and mouth are involved in approximately 80 % of cases [22]. Ocular involvement is estimated to occur in around 50 % of patients [23]. Both eyes are usually involved, although it may be asymmetrical. Ocular symptoms include eye pain, redness, itching, burning, dry eye, foreign body sensation and mucus discharge. Ocular findings may be clinically indistinguishable from those in OcMMP. Slit lamp examination may reveal conjunctival subepithelial fibrosis and subsequent loss of inner canthal architecture, fornix foreshortening and symblepharon formation. Other findings include conjunctival bullae, entropion, trichiasis, corneal opacification and blindness [24, 25].

EBA is a very rare subepidermal blistering disorder characterised by IgG autoantibodies to type VII collagen, a primary component of the BMZ anchoring fibril [26–29]. Circulating antibodies are seen in 20–50 % of patients with EBA, and IgA antibodies are seen even less frequently [30]. In some patients with EBA, IgA antibodies are more abundant than those of the IgG class or are the only immunoglobulin class detected—this is termed “IgA-EBA” [31–33].

Mucosal involvement in EBA is common and primarily oral [22]. Ocular manifestations include bilateral subepithelial vesicles, conjunctival erosion, conjunctival scarring, blepharitis and cicatricial trichiasis. Corneal abnormalities include peripheral ulcerative keratitis, corneal neovascularisation, corneal thinning, corneal perforation, corneal scarring, corneal pannus (Fig. 45.3) and opacification [22, 24, 34]. The IgA-EBA form of the disease is associated with more severe ocular involvement [31–33], with total blindness reported to occur in these patients [35, 36].

Ocular complications are seen less commonly in the other types of autoimmune blistering diseases.

In pemphigus vulgaris, the mucous membranes are usually the first affected by the disease. Erosions in the oral cavity occur commonly. The mucosal surfaces including the conjunctiva, oesophagus and genital areas can also be affected, but are less common. Ocular involvement in pemphigus is rare [11]. It may however precede disease manifestations at other sites [37]. Ocular disease can present with symptoms of ocular irritation, tearing and foreign body sensation [37–39]. Ocular findings reported include conjunctivitis with mucoid discharge, conjunctival oedema and conjunctival vesicles [40]. In a case series of 11 patients with PV, Daoud et al. reported ocular findings of bilateral conjunctivitis and lid margin ulceration. The mean interval between onset of PV and ocular involvement was 20 months, and the ocular disease lasted for a mean of 12 months. The authors found that patients had complete recovery and no long-term sequelae due to conjunctival disease. Based on their cohort of 167 patients with PV, Daoud et al. estimated an incidence of ocular involvement in pemphigus vulgaris of 7 %. Fornix foreshortening and plica semilunaris vegetations have also been reported by other authors in the literature [41]. Ocular disease does not appear to affect visual acuity, and patients usually have a full recovery without long-term complications [42]. Pemphigus foliaceus, a separate type of pemphigus, may produce entropion and trichiasis of both eyelids with resultant corneal damage (Fig. 45.4). Progressive scarring and blindness usually do not occur [43].

Mucous membranes are involved only occasionally in bullous pemphigoid; consequently, ocular involvement is uncommon. Ocular manifestations are subtle and may go undetected in the conjunctiva and lid margins. Slit lamp examination may reveal subepithelial fibrosis with fine linear striae of the tarsus [44, 45]. Other ocular manifestations reported in the literature include conjunctival scarring, corneal epithelial defects, stroma oedema and corneal opacity [44, 45]. Corneal melt [46] and filamentary keratitis [47] have been reported in paraneoplastic pemphigus. In dermatitis herpetiformis, ophthalmic involvement is rare and is limited to the periorbital skin [48].

Treatment in autoimmune blistering skin diseases is individualised based on age, disease severity and the sites of involvement in the patient. In patients who present with early-stage disease, topical corticosteroids such as triamcinolone acetonide, fluocinonide or clobetasol propionate may be used. Topical corticosteroids are however inadequate to halt disease progression. Systemic chemotherapy is used for acute exacerbations and in chronic disease. Dapsone is used in milder cases unless the patient has a history of glucose-6-phosphate dehydrogenase deficiency or drug intolerance (Refs. [35, 36] in Elchahal). In moderate to severe cases, cytotoxic agents are used to suppress conjunctival inflammation and cicatrisation. These agents include mycophenolate mofetil, sulphasalazine, methotrexate and azathioprine. Cyclophosphamide together with prednisolone at a dose of 1 mg per 1 kg of body weight per day is used in resistant cases. In patients whose disease is refractory to immunosuppressive therapy, intravenous immunoglobulin (IVIG) can be used. It is administered at a dose of 2–3 g per 1 kg of body weight per cycle, with each cycle lasting for three to five consecutive days each month. Adverse events associated with IVIG therapy are usually mild and self-limiting. The use of IVIG therapy is however limited due to its high cost. If the cost of management of side effects and hospitalisations are included however, IVIG has been shown to be more cost-effective than immunosuppressive therapy over the entire disease course and an on annual basis. Etanercept and infliximab are other treatment options in disease refractory to corticosteroids or immunosuppressive drugs. Surgical techniques are used in cases of MMP with advanced corneal keratinisation or ulceration. These include amniotic membrane grafting, penetrating keratoplasty and limbal stem cell transplantation. If these techniques are unsuccessful, keratoprostheses may be another option to regain useful vision in the affected eye.