KEGG   DISEASE: Cutaneous lupus erythematosus
Entry
H01595                      Disease                                
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
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H01595  Cutaneous lupus erythematosus
 Skin diseases
  Skin and soft tissue diseases
   H01595  Cutaneous lupus erythematosus
Human diseases in ICD-11 classification [BR:br08403]
 14 Diseases of the skin
  Inflammatory dermatoses
   Cutaneous lupus erythematosus
    EB50  Subacute cutaneous lupus erythematosus
     H01595  Cutaneous lupus erythematosus
    EB51  Chronic cutaneous lupus erythematosus
     H01595  Cutaneous lupus erythematosus
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
  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
Reference
  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
Reference
PMID:26850655 (drug)
  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
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