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Annales de Dermatologie et de Vénéréologie
Volume 137, n° 8-9
pages 541-545 (août 2010)
Doi : 10.1016/j.annder.2010.04.015
Received : 25 November 2009 ;  accepted : 1 April 2010
Une vascularite hypocomplémentémique traitée par dapsone
Hypocomplementemic vasculitis treated with dapsone
 

M. Hérault a, J. Mazet b, P. Beurey c, J.-F. Cuny a, A. Barbaud a, J.-L. Schmutz a, A.-C. Bursztejn a,
a Service de dermatologie, hôpital Fournier, CHU de Nancy, 36, quai de la bataille, 54000 Nancy, France 
b Cabinet de dermatologie, 15, rue Isabey, 54000 Nancy, France 
c Cabinet d’anatomopathologie, 5, rue Isabey, 54000 Nancy, France 

Auteur correspondant.
Résumé
Introduction

La vascularite urticarienne hypocomplémentémique de Mac Duffie, décrite en 1973, est une entité rare dont l’individualisation fait débat. Nous rapportons un cas de vascularite de Mac Duffie d’évolution favorable sous dapsone.

Observation

Un homme de 43 ans présentait, depuis plusieurs années, des poussées de lésions urticariennes accompagnées d’œdèmes palpébraux et de signes généraux tels que fièvre et arthralgies. En 2006, le diagnostic de vascularite urticarienne de Mac Duffie était porté devant une hypocomplémentémie et la présence d’anticorps anti-C1q. Un traitement par dapsone était institué, permettant une amélioration de la symptomatologie.

Discussion

La vascularite urticarienne de Mac Duffie associe des lésions urticariennes évoluant par poussées à une image histologique de vascularite leucocytoclasique et à des manifestations systémiques. Ces dernières, proches des manifestations du lupus, font discuter l’appartenance du syndrome de Mac Duffie au lupus érythémateux systémique (LES). Le diagnostic repose sur la clinique, l’anatomopathologie et la présence d’anticorps anti-C1q. Le traitement n’est pas codifié ; en cas d’atteinte sévère, les immunosuppresseurs sont proposés après échec de la corticothérapie générale.

The full text of this article is available in PDF format.
Summary
Background

Hypocomplementemic urticarial vasculitis, described by MacDuffie in 1973, is rare. Some doubt surrounds its classification. We report a case of hypocomplementemic urticarial vasculitis (MacDuffie syndrome) treated with dapsone with a favorable outcome.

Case report

Over a number of years, a 43-year-old man presented urticarial vasculitis attacks with palpebral oedema and systemic symptoms such as fever and arthralgia. In 2006, MacDuffie syndrome was diagnosed on the grounds of positive anti-C1q antibodies. Treatment with dapsone was started and resulted in considerable improvement.

Discussion

Hypocomplementemic urticarial vasculitis is characterized by urticarial vasculitis lesions, leucocytoclastic vasculitis and systemic symptoms. The latter symptoms are similar to those of systemic lupus erythematosus (SLE), and some authors have suggested that MacDuffie syndrome may in fact belong to SLE. Diagnosis is based on clinical appearance, histology and the presence of anti-C1q antibodies. There is no specific treatment for hypocomplementemic urticarial vasculitis. Immunosuppressant therapy can be used for lesions refractory to systemic corticosteroids.

The full text of this article is available in PDF format.

Mots clés : Vascularite urticarienne, Mac Duffie, Dapsone

Keywords : Urticarial vasculitis, MacDuffie, Dapsone




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