Letters to the Editor

Unilateral ocular cicatricial pemphigoid with circulating IgA and IgG autoantibodies reactive with the 180 kD bullous pemphigoid antigen EDrroR,-Cicatricial pemphigoid (CP) is a rare, chronic, vesiculobullous disease that primarily affects the mucous membranes, particularly of the mouth and eyes.' This disease is attributed to a subepidermal autoimmune phenomenon, and is characterised by in vivo deposition of antiepithelial basement membrane zone (BMZ) antibodies.' Fibrosis in ocular cicatricial pemphigoid (OCP) produces shrinkage of the conjunctiva followed by shortening of the fornices, symblepharon, and cicatricial entropion. The disease usually affects both eyes. We present a patient with OCP in one eye, who exhibited circulating IgA and IgG auto-antibodies. These antibodies bound to a 180 kD antigen resembling that recognised by sera from patients with bullous pemphigoid (BP). Circulating IgA antibodies against the 180 kD BP antigen have not been reported in a patient with CP. A 60-year-old Japanese man was referred to our hospital with redness, discharge, and irritation of the right eye that have been present for about 1 year. Physical examination revealed conjunctival injection, shortening of the fornix, symblepharon, and superficial punctate keratitis due to entropion of his right eye, while the left eye appeared normal. Impression cytology indicated a scarcity of goblet cells in the right conjunctiva but a normal amount in the left conjunctiva. Erosions Figure 1 Direct immunofluorescence demonstrates linear deposition ofIgA along the epithelial basement membrane zone. Figure 2 Indirect immunofluorescence using I M sodium chloride-split skin showed that IgA antibodies in this patient's serum reacted with the epidermal side of the split. were observed on the oral and nasal mucosa but there were no skin lesions. A direct immunofluorescence was performed on a biopsy specimen of the right con-junctiva and revealed linear deposits of IgG, IgA, and C3 along the epithelial BMZ (Fig 1). An indirect immunofluorescence for identifying IgG and IgA antibodies in the patient's serum was performed using 1 M sodium chloride-split skin, described by Gammon et al.3 Both IgG and IgA antibodies in the serum reacted with the epidermal side of the split (Fig 2). To identify the BMZ antigens, western blotting was performed as described previously.4 IgG in the patient's serum reacted relatively weakly but clearly with antigens with molecular weights of approximately 230 kD and 180 kD, and IgA reacted only with the 180 kD antigen (Fig 3). The 230 kD antigen co-migrated with the 230 kD BP antigen, while the …