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June 2006

Ocular Linear IgA Disease Resulting in Blindness

Arch Dermatol. 2006;142(6):781-798. doi:10.1001/archderm.142.6.786

Linear IgA disease (LAD) is an autoimmune subepidermal bullous disorder that may affect skin and/or mucous membranes. The presence of ocular involvement as the only manifestation of the disease is very rare.1 We report a case of LAD that was limited to the eyes and progressed to bilateral blindness.

A 75-year-old man with a 4-year history of eye disease presented in August 2000 with possible autoimmune blistering disease. Therapy with topical antibiotics and systemic corticosteroids had been ineffective. He stated that he had experienced redness, a burning sensation, and a reduction of visual acuity in his eyes over the past year. Ophthalmologic examination revealed conjunctival shrinking and scarring, cicatricial entropion, athrichiasis, and conjunctival injection (Figure 1), as well as symblepharon in the right lower fornix and erosion of the left cornea. The patient's skin, oral cavity, and genitalia were normal. Conjunctival biopsy revealed a subepithelial blister with fibrosis and an infiltrate composed of lymphocytes, neutrophils, and eosinophils. Direct immunofluorescence showed linear deposition of IgA along the basement membrane zone. The results of indirect immunofluorescence of normal and 1-mol/L sodium chloride–split human skin were negative. The diagnosis of LAD was made, and treatment with mycophenolate mofetil (2 g/d) and prednisone (60 mg/d) was initiated. Two months later, severe pain developed in the patient's left eye. The pain was found to be caused by a corneal perforation. Corneal transplantation with limbal stem cells was performed, and cyclosporin (150 mg/d) was added to the treatment regimen. Four months later, transplantation rejection caused redness and deterioration of visual acuity in the left eye. The patient refused further therapy. In February 2004, he presented with a 3-year history of untreated disease. He had complete scarring and shrinkage of the conjunctival fornices of the right eye and could no longer open his eyelids (Figure 2). He had only partial light perception in his left eye.