A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens
✍ Scribed by P. Musette; P. Joly; D. Gilbert; E. Marinho; B. Crickx; F. Tron; S. Belaich; V. Descamps
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 367 KB
- Volume
- 143
- Category
- Article
- ISSN
- 0007-0963
No coin nor oath required. For personal study only.
✦ Synopsis
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.