Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions

Citation
D. Lipsker et al., Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions, BR J DERM, 142(5), 2000, pp. 954-959
Citations number
21
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Dermatology,"da verificare
Journal title
BRITISH JOURNAL OF DERMATOLOGY
ISSN journal
0007-0963 → ACNP
Volume
142
Issue
5
Year of publication
2000
Pages
954 - 959
Database
ISI
SICI code
0007-0963(200005)142:5<954:SSHIFI>2.0.ZU;2-3
Abstract
The Schnitzler syndrome is the association of chronic urticaria, intermitte nt fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy, It i s not yet firmly established whether the monoclonal immunoglobulin componen t plays a part in the pathophysiology of the urticarial lesions. Immunoblot ting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were perfo rmed in three patients with the Schnitzler syndrome. Western blotting on ep idermal extracts showed the presence of IgM-kappa antiskin autoantibodies i n two patients, These antibodies displayed the same isotype as the monoclon al components and recognized a 280-290-kDa antigen in one patient and a 100 -kDa antigen in the other patient. IEM studies showed sparse IgM deposits i n the epidermis, around the keratinocytes, near the desmosomes in one patie nt and dense deposits below the lamina densa, in the region of the anchorin g fibrils, in another patient. Antiskin IgM autoantibodies of the same isot ype as their monoclonal gammopathies can be present in the serum of some pa tients with the Schnitzler syndrome, These IgM antibodies seem to deposit i n vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammo pathy prays a part in the pathophysiology of the skin rash, They also sugge st patient heterogeneity both in the skin antigens that are recognized as w ell as in their localization.