Premium
A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens
Author(s) -
Musette P.,
Joly P.,
Gilbert D.,
Marinho E.,
Crickx B.,
Tron F.,
Belaich S.,
Descamps V.
Publication year - 2000
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.1365-2133.2000.03605.x
Subject(s) - antigen , medicine , disease , dermatology , immunology , pathology
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.