title: A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.
abstract: 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.
authors.affiliation: Departments of Dermatology, Hôpital Bichat, 46 rue H. Huchard, 75018 Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Musette
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: D
authors.initials: D
authors.lastname: Gilbert
authors.forename: E
authors.initials: E
authors.lastname: Marinho
authors.forename: B
authors.initials: B
authors.lastname: Crickx
authors.forename: F
authors.initials: F
authors.lastname: Tron
authors.forename: S
authors.initials: S
authors.lastname: Belaich
authors.forename: V
authors.initials: V
authors.lastname: Descamps
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D016393:Lymphoma, B-Cell / Q000150:complications / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000209:etiology / Q000276:immunology; D011230:Precancerous Conditions / Q000150:complications / Q000276:immunology*; D012872:Skin Diseases, Vesiculobullous / Q000150:complications / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D007075:Immunoglobulin M