H01595 | |
H number | H01595 |
Name | Cutaneous lupus erythematosus |
Description | Cutaneous lupus erythematosus (CLE) is the skin-related form of lupus erythematosus (LE), with a broad spectrum of clinical manifestations and a variable course. CLE is a frequent finding in patients with systemic lupus erythematosus (SLE) and can also exist as a single entity without associated systemic autoimmunity (LE-specific skin disease). Despite ongoing research into the cause of CLE, it remains unclear how CLE relates to SLE pathogenesis. LE-specific skin disease includes the subtypes of CLE such as acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE), and intermittent CLE (ICLE). ACLE is characterized by malar rash or maculopapular eruption on sun exposed areas. SCLE characteristically presents as annular or psoriasiform plaques in a photo distribution. CCLE can be further divided into discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain LE (CLE), and LE tumidus (LET). The pathogenesis of CLE is multifactorial and involves genetic predisposition, environmental triggers, and abnormalities in the immune response. It has been reported that many genetic risk factors for CLE involve HLA or interferon-related pathways. Corticosteroids and antimalarials were considered as the most suitable and effective systemic drugs for CLE patients. |
Category | Immune system disease; Skin disease |
Network | - |
Gene | - |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Triamcinolone hexacetonide [DR:D00985] Methylprednisolone acetate [DR:D00979] Hydroxychloroquine sulfate [DR:D02114] |
Comment | See also H00080 Systemic lupus erythematosus, and H00291 Familial chilblain lupus (FCL). |
Other DBs | ICD-11: EB50 EB51 ICD-10: L93 MeSH: D008178 |
Reference | PMID:27270345 AUTHORS Stannard JN, Kahlenberg JM TITLE Cutaneous lupus erythematosus: updates on pathogenesis and associations with systemic lupus. JOURNAL Curr Opin Rheumatol 28:453-9 (2016) DOI:10.1097/BOR.0000000000000308 PMID:18042093 AUTHORS Kuhn A, Sticherling M, Bonsmann G TITLE Clinical manifestations of cutaneous lupus erythematosus. JOURNAL J Dtsch Dermatol Ges 5:1124-37 (2007) DOI:10.1111/j.1610-0387.2007.06554.x PMID:26850655 AUTHORS Chasset F, Arnaud L, Costedoat-Chalumeau N, Zahr N, Bessis D, Frances C TITLE The effect of increasing the dose of hydroxychloroquine (HCQ) in patients with refractory cutaneous lupus erythematosus (CLE): An open-label prospective pilot study. JOURNAL J Am Acad Dermatol 74:693-699.e3 (2016) DOI:10.1016/j.jaad.2015.09.064 |