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[Antidesmoglein antibodies in a patient with Hailey-Hailey disease]
I Bennani1, J Ofaiche, C Uthurriague
1Service de dermatologie, centre hospitalier de Montauban, 100, rue Léon-Cladel, 82013 Montauban cedex, France.
Background:
Hailey-Hailey disease (HHD) is a rare hereditary disease in which the genetic defect is characterized by mutation in the ATP2C1 gene coding for a transmembrane calcium pump. It is generally considered a non-immunologic acantholytic dermatosis in which direct and indirect immunofluorescence studies are negative, unlike in autoimmune pemphigus.
Patients And Methods:
We describe a case of HHD associated with antidesmoglein antibodies in a 53-year-old woman. The clinical symptoms and histology were typical of HHD. Antidesmoglein antibody tests were positive on several occasions and a difference was found between the two types of Elisa test performed (positive with the MBL kit, negative with the Euroimmun kit).
Discussion:
The positive result for desmoglein antibodies could be due to unmasking of antigens by the mechanism of acantholysis. The specificity of the main desmoglein Elisa tests also requires discussion.
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