A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.
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.
Musette
P
P
Departments of Dermatology, Hôpital Bichat, 46 rue H. Huchard, 75018 Paris, France.
Joly
P
P
Gilbert
D
D
Marinho
E
E
Crickx
B
B
Tron
F
F
Belaich
S
S
Descamps
V
V
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*
D001323:Autoantibodies; D007074:Immunoglobulin G; D007075:Immunoglobulin M