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Ocular involvement in IgA–epidermolysis bullosa acquisita
Author(s) -
Bauer J.W.,
Schaeppi H.,
Metze D.,
Muss W.,
PohlaGubo G.,
Hametner R.,
Ruckhofer J.,
Grabner G.,
Hintner H.
Publication year - 1999
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1999.03163.x
Subject(s) - epidermolysis bullosa acquisita , medicine , dermatology , anchoring fibrils , lamina densa , epidermolysis bullosa , lamina lucida , pathology , conjunctiva , cicatricial pemphigoid , milia , pemphigoid , autoantibody , basement membrane , bullous pemphigoid , immunology , antibody , ultrastructure , basal lamina
Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disease with frequent ocular involvement, but visual loss is rare. In contrast, EBA patients with predominant IgA autoantibodies more frequently develop severe ocular involvement, which tends to be refractory to therapy. We report two patients with ‘IgA–EBA’ with ocular involvement. Both initially presented with a generalized bullous disease, and direct immunofluorescence microscopy demonstrated IgA in the basement membrane zone of the skin, and in the conjunctiva and cornea of patient 1. On salt‐split patient skin, IgA was found predominantly on the dermal side of the artificial split in both patients. Direct immunoelectron microscopy demonstrated IgA below the lamina densa in close association with the anchoring fibrils in both patients. In patient 1, who had a prolonged course of the disease, the skin disorder responded well to treatment with cyclosporin, but the ocular involvement ended in bilateral blindness despite repeated surgical treatment. In patient 2, the blister formation and scarring conjunctivitis was stopped by a combination of prednisolone and colchicine. These patients show that in subepithelial blistering diseases, early delineation of disease nosology is critical to detect subtypes with severe ocular involvement such as ‘IgA–EBA’. In addition, colchicine may be a valuable alternative in the treatment of EBA with ocular involvement.

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