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A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens

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We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient’s clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I–II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.
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Keywords: bullous skin disease; lymphoma; paraneoplastic skin disease; tolerance

Document Type: Research Article

Affiliations: 1: Departments of Dermatology and 2: Unité INSERM US19 et IFR 23, Hôpital Charles Nicolle, Rouen, France 3: Pathology, Hôpital Bichat, 46 rue H.Huchard, 75018 Paris, France

Publication date: 2000-07-01

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