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

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Betul Sozeri ◽  
Sevgi Mir ◽  
Afig Berdeli
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Cutaneous Lupus Erythematosus ◽  
Classical Pathway ◽  
Systemic Lupus ◽  
Resistant Disease ◽  
Complement 4 ◽  
Genetically Determined ◽  
Cutaneous Lupus

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.

scholarly journals Cutaneous Manifestations of “Lupus”: Systemic Lupus Erythematosus and Beyond

2021 ◽  
Vol 2021 ◽  
pp. 1-19
Author(s):  
Elizabeth E. Cooper ◽  
Catherine E. Pisano ◽  
Samantha C. Shapiro
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Treatment ◽  
Treatment Options ◽  
Cutaneous Lupus Erythematosus ◽  
Laboratory Studies ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Distinct Disease ◽  
Cutaneous Lupus

Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.

Cutaneous lupus erythematosus and systemic lupus erythematosus are associated with clinically significant cardiovascular risk: a Danish nationwide cohort study

Lupus ◽  
2016 ◽  
Vol 26 (1) ◽  
pp. 48-53 ◽  
Author(s):  
J Halskou Hesselvig ◽  
O Ahlehoff ◽  
L Dreyer ◽  
G Gislason ◽  
K Kofoed
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cohort Study ◽  
Cardiovascular Risk ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Low Grade ◽  
Systemic Lupus ◽  
Increased Risk ◽  
All Cause Mortality ◽  
Cutaneous Lupus

Systemic lupus erythematosus (SLE) is a well-known cardiovascular risk factor. Less is known about cutaneous lupus erythematosus (CLE) and the risk of developing cardiovascular disease (CVD). Therefore, we investigated the risk of mortality and adverse cardiovascular events in patients diagnosed with SLE and CLE. We conducted a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular endpoint and all-cause mortality, for patients with SLE and CLE. A total of 3282 patients with CLE and 3747 patients with SLE were identified and compared with 5,513,739 controls. The overall HR for the composite CVD endpoint was 1.31 (95% CI 1.16–1.49) for CLE and 2.05 (95% CI 1.15–3.44) for SLE. The corresponding HRs for all-cause mortality were 1.32 (95% CI 1.20–1.45) for CLE and 2.21 (95% CI 2.03–2.41) for SLE. CLE and SLE were associated with a significantly increased risk of CVD and all-cause mortality. Local and chronic inflammation may be the driver of low-grade systemic inflammation.

The study of Cutaneous Lupus Erythematosus Disease Area and Severity Index in Indian patients with systemic lupus erythematosus

Lupus ◽  
2011 ◽  
Vol 20 (14) ◽  
pp. 1510-1517 ◽  
Author(s):  
P Salphale ◽  
D Danda ◽  
L Chandrashekar ◽  
D Peter ◽  
N Jayaseeli ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Severity Index ◽  
Skin Lesions ◽  
Systemic Lupus ◽  
Disease Area ◽  
Indian Patients ◽  
The Mean ◽  
Cutaneous Lupus

The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a newly described tool used to assess the activity of and damage caused by cutaneous lupus erythematosus (CLE). There is a paucity of data on CLASI from the Indian subcontinent. We sought to determine the applicability of CLASI in specific lesions of CLE in patients with systemic lupus erythematosus (SLE) attending a tertiary care hospital in India. In this prospective, cross-sectional study, 93 patients of SLE with cutaneous lesions were recruited. CLASI activity and damage scores of lupus erythematosus (LE)-specific skin lesions were done in 75 patients with SLE. The mean CLASI activity score was 15.4 ± 9.4 (range 0–39) and the mean damage score was 6.87 ± 7.75 (range 0–30). Higher mean CLASI activity scores were seen in patients with a combination of acute, subacute and chronic CLE and in those with widespread lesions. Patients with longstanding disease and long duration of skin lesions had higher damage scores. This study shows that CLASI is an effective tool to assess cutaneous activity of LE-specific lesions, and the damage caused by them, in Indian patients.

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