Table of Contents
ISRN Rheumatology
Volume 2011, Article ID 917673, 5 pages
http://dx.doi.org/10.5402/2011/917673
Case Report

Complement-4 Deficiency in a Child with Systemic Lupus Erythematosus Presenting with Standard Treatment-Resistant Severe Skin Lesion

1Department of Pediatric Rheumatology, Faculty of Medicine, Ege University, Bornova, 35040 Izmir, Turkey
2Department of Pediatric Nephrology, Faculty of Medicine, Ege University, Bornova, 35040 Izmir, Turkey
3Department of Molecular Medicine, Faculty of Medicine, Ege University, Bornova, 35040 Izmir, Turkey

Received 4 December 2010; Accepted 10 January 2011

Academic Editor: M. Goto

Copyright © 2011 Betul Sozeri et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The complement system is of great importance in systemic lupus erythematosus. Complete genetically determined deficiencies are with few exceptions reported for the various complement proteins, and most of the deficiency states are rare. Deficiencies of the factors in the classical pathway are also associated with development SLE and SLE-like disorders. Most of the patients with lupus present skin involvement. Approximately, 75–95% of patients with cutaneous lupus erythematosus respond to antimalarial therapy and/or topical glucocorticosteroids. Immunosuppressive agents are usually considered a second-line approach in patients with resistant disease. In this study, we present the clinical features and determine the molecular basis responsible for the complete C4A and C4B deficiencies in a lupus patient presented subacute cutaneous lupus erythematosus and resistance to treatment.