Skip to main content
padlock icon - secure page this page is secure

Schnitzler syndrome: heterogeneous immunopathologicalfindings involving IgM–skin interactions

Buy Article:

$52.00 + tax (Refund Policy)

The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-κ antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280–290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal–epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.
No References
No Citations
No Supplementary Data
No Article Media
No Metrics

Keywords: IgM monoclonal gammopathy; Schnitzler syndrome; immunoblotting; immunoelectron microscopy

Document Type: Research Article

Affiliations: 1: EPI INSERM 99-08, Établissement de Transfusion Sanguine, 10 rue Spielmann, F-67065 Strasbourg cedex, France 2: Laboratoire d’Immunopathologie, Centre Hospitalier, 4 rue Barblé, L-1210 Luxembourg, Luxembourg 3: Laboratoire d’Immunopathologie de la Clinique Dermatologique des Hôpitaux Universitaires, 1 place de l’Hôpital, F-67091 Strasbourg cedex, France

Publication date: May 1, 2000

  • Access Key
  • Free content
  • Partial Free content
  • New content
  • Open access content
  • Partial Open access content
  • Subscribed content
  • Partial Subscribed content
  • Free trial content
Cookie Policy
Cookie Policy
Ingenta Connect website makes use of cookies so as to keep track of data that you have filled in. I am Happy with this Find out more