II. Classification: Chronic Cutaneous Lupus (CCLE)

  1. Discoid Lupus Erythematosus (DLE)
    1. Most common form of Chronic Cutaneous Lupus
    2. Peak Incidence in ages 30 to 40 years
    3. More common in women
    4. Exacerbated by Trauma and sunlight
    5. Asymmetric red 1-2 cm Plaques with adherent scale on the face and scalp, neck and ears
      1. Progresses to local skin atrophy over months
      2. Ultimately results in scar
        1. Scar with altered pigmentation (Hypopigmentation, Hyperpigmentation)
        2. Usually depressed scar, but may be hypertrophic
      3. Several presentation variants
        1. Generalized variant also involves trunk and extremities
        2. Hypertrophic variant
  2. Subacute Cutaneous Lupus (SCLE)
    1. Mid-category between CCLE and ACLE
    2. May be associated with Systemic Lupus Erythematosus or a drug-induced event
    3. Usually heals without scarring (unlike CCLE)
    4. Annular erythematous Plaques (psoriaform)
      1. Distributed over Shoulders and Forearms, neck and upper trunk
      2. Central Hypopigmentation and Telangiectasia
  3. Acute Cutaneous Lupus (ACLE)
    1. Occurs in combination with Systemic Lupus Erythematosus in most cases
    2. Malar Rash or Butterfly Rash (most common skin manifestation in ACLE)
      1. Erythema over the cheeks and bridge of nose
    3. Red Plaques
      1. Occurs on face, chest, and Shoulders
      2. Extensor arm and hand dorsum also involved
    4. Other skin manifestations
      1. Toxic Epidermal Necrolysis type skin eruption

III. Differential Diagnosis

IV. Labs

  1. Skin Biopsy
    1. Consider ordering immunofluorescence for older lesion
  2. Serologic Testing if biopsy suggests Cutaneous Lupus
    1. ANA titer (positive if 1:160 or higher)
      1. Chronic Cutaneous Lupus (CCLE): 4%
      2. Subacute Cutaneous Lupus (SCLE): 63%
      3. Acute Cutaneous Lupus (ACLE): 98%
    2. Anti-dsDNA
      1. Subacute Cutaneous Lupus (SCLE): 30%
      2. Acute Cutaneous Lupus (ACLE): 60-80%
    3. Complement (C3 or CH50)
      1. Suppressed in 90% of Acute Cutaneous Lupus (ACLE)
    4. Anti-Ro Antibody (Anti-SSA)
      1. Associated with Subacute Cutaneous Lupus (SCLE)
  3. Further evaluation
    1. See Systemic Lupus Erythematosus
    2. Distinguish systemic from isolated skin involvement

V. Management

  1. Sunscreen with UVA and UVB protection is critical
  2. Non-systemic agents
    1. Topical Corticosteroids (first choice)
    2. Calcineurin Inhibitors (e.g. Tacrilimus)
    3. Physical treatments
      1. Laser therapy
      2. Cryotherapy
      3. Dermabrasion
    4. Intralesional Corticosteroid
      1. Indicated for Discoid Lupus (DLE, CCLE)
  3. Systemic agents
    1. First-line: Hydroxychloroquine (Plaquenil)
    2. Other agents (if refractory to topicals, Plaquenil)
      1. Dapsone
      2. Oral Corticosteroids
      3. Azathioprine
      4. Isotretinoin
      5. Methotrexate

VI. References

  1. Merola (2019) UpToDate, accessed 12/4/2019
  2. Habif (1996) Dermatology, p. 544-9
  3. Okon (2013) Best Pract Res Clin Rheumatol 27(3): 391–404. [PubMed]
  4. Patel (2002) Dermatol Clin 20(3):373-85 [PubMed]

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Related Studies

Ontology: Barrett Esophagus (C0004763)

Definition (NCI_NCI-GLOSS) A condition in which the cells lining the lower part of the esophagus have changed or been replaced with abnormal cells that could lead to cancer of the esophagus. The backing up of stomach contents (reflux) may irritate the esophagus and, over time, cause Barrett's esophagus.
Definition (NCI) Esophageal lesion lined with columnar metaplastic epithelium which is flat or villiform. Barrett epithelium is characterized by two different types of cells: goblet cells and columnar cells. The symptomatology of Barrett esophagus is that of gastro-esophageal reflux. It is the precursor of most esophageal adenocarcinomas. (WHO)
Definition (MSH) A condition with damage to the lining of the lower ESOPHAGUS resulting from chronic acid reflux (ESOPHAGITIS, REFLUX). Through the process of metaplasia, the squamous cells are replaced by a columnar epithelium with cells resembling those of the INTESTINE or the salmon-pink mucosa of the STOMACH. Barrett's columnar epithelium is a marker for severe reflux and precursor to ADENOCARCINOMA of the esophagus.
Definition (CSP) syndrome including peptic ulcer of the lower esophagus, often with stricture, due to the presence of columnar lined epithelium, which may contain functional mucous cells, parietal cells, or chief cells in the esophagus instead of normal squamous cell epithelium; sometimes pre-malignant, followed by esophageal adenocarcinoma.
Concepts Disease or Syndrome (T047)
MSH D001471
ICD9 530.85
ICD10 K22.7 , K22.70
SnomedCT 235597001, 196609006, 302914006, 76355008, 155679007, 196603007
LNC LA14288-7
English Esophagus, Barrett, Syndrome, Barrett's, CELLO - Col epith-lin low oeso, CLE - Columnar-lined oeso, Columnar epith-lined low oeso, BO - Barrett's esophagus, Barrett's Esophagus, Barretts Esophagus, Esophagus, Barrett's, Barrett Syndrome, Barrett's Syndrome, Barretts Syndrome, Syndrome, Barrett, Barrett's esophagus (diagnosis), Barrett's oesophagitis, Barrett esophagus, Barrett Esophagus [Disease/Finding], barrett's esophagitis, barrett's esophagus, barretts syndrome, barrett's oesophagitis, barrett's oesophagus, barrett esophagus, barrett's syndrome, barrett syndrome, barrett oesophagitis, barrett esophagitis, Barretts syndrome, Barrett's esophagus NOS, Barrett's disease, Endobrachyoesophagus, BARRETT ESOPHAGUS, BARRETT METAPLASIA, Barretts esophagus, Barrett's esophagitis, Barrett Metaplasia, Barrett's oesophagus (disorder), Endobrachyooesophagus, Columnar-lined esophagus, Barrett's esophagus, Barrett's syndrome, Gastric metaplasia of esophagus, Barrett's oesophagus, Columnar-lined oesophagus, Gastric metaplasia of oesophagus, BO - Barrett's oesophagus, CELLO - Columnar epithelial-lined lower esophagus, CELLO - Columnar epithelial-lined lower oesophagus, CLE - Columnar-lined esophagus, CLE - Columnar-lined oesophagus, Columnar epithelial-lined lower esophagus, Columnar epithelial-lined lower oesophagus, Barrett's esophagus (disorder), Barrett, Barrett's esophagus (disorder) [Ambiguous], Barrett Esophagus, BE, Columnar Epithelial-Lined Lower Esophagus, Columnar-Lined Esophagus, CELLO, CLE, Esophagitis;Barretts, Oesophagitis;Barretts, Barretts esophagitis, Barretts oesophagitis
Dutch Barret-oesophagus, endobrachyoesophagus, Barrett-oesophagitis, Barrett-oesofagitis, Barrett-oesophagus, Barrett-oesofagus, Barrett-syndroom, Oesofagus, Barrett-
French Oesophagite de Barrett, EBO (EndoBrachyOesophage), Endobrachyoesophage, Oesophage de Barrett, Syndrome de Barrett
German Barrett-Oesophagitis, Endobrachyoesophagus, Barrett-Ulkus, Barrett-Syndrom, Barrett-Ösophagus, Ösophagus, Barrett-
Italian Endobrachiesofago, Esofagite di Barrett, Sindrome di Barrett, Esofago di Barrett
Portuguese Endobraquiesófago, Esofagite de Barrett, Esófago de Barrett, Esôfago de Barrett, Síndrome de Barrett
Spanish Endobraquioesófago, Esofagitis de Barrett, síndrome de Barret, epitelio cilíndrico en el esófago distal, esófago distal revestido con epitelio cilíndrico, esófago con epitelio columnar, síndrome de Barrett, metaplasia gástrica de esófago, epitelio columnar en el esófago distal, esófago de Barret (concepto no activo), esófago de Barret (trastorno), esófago de Barret, metaplasia gástrica del esófago, esófago de Barret [dup] (trastorno), Esófago de Barrett, Síndrome de Barrett
Japanese 食道円柱上皮化, バレットショクドウ, ショクドウエンチュウジョウヒカ, Barrett食道, Barrett症候群, バレット食道, バレット症候群, 食道-バレット
Swedish Barretts esofagus
Finnish Barrettin ruokatorvi
Czech Endobrachyezofagus, Barretův jícen, Barrettův jícen, Barrettův ezofágus, Barretova ezofagitida
Korean 바렛 식도
Polish Wrzód trawienny przełyku, Przełyk Barretta
Hungarian Barrett oesophagitis, Endobrachyoesophagus, Barrett-oesophagus, Barrett oesophagus, Barrett-oesophagitis
Norwegian Barretts syndrom, Barretts sykdom, Barretts øsofagus, Barretts oesophagus

Ontology: Lupus Erythematosus, Discoid (C0024138)

Definition (MSH) A chronic form of cutaneous lupus erythematosus (LUPUS ERYTHEMATOSUS, CUTANEOUS) in which the skin lesions mimic those of the systemic form but in which systemic signs are rare. It is characterized by the presence of discoid skin plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy. Lesions are surrounded by an elevated erythematous border. The condition typically involves the face and scalp, but widespread dissemination may occur.
Definition (CSP) chronic form of cutaneous lupus erythematosus in which the skin lesions mimic those of the systemic form but in which systemic signs are rare; characterized by the presence of discoid skin plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy; lesions are surrounded by an elevated erythematous border; the condition typically involves the face and scalp, but widespread dissemination may occur.
Concepts Disease or Syndrome (T047)
MSH D008179
ICD10 L93.0
SnomedCT 156365002, 13902000, 200938002, 238927000
LNC LA15302-5
English Lupus Erythematosus, Chronic Cutaneous, Lupus Erythematosus, Discoid, Lupus Erythematosus, Cutaneous, Chronic, DLE, LUPUS ERYTHEMATOSUS DISCOID, DLE - Discoid lupus erythemat, LE-Discoid lupus erythematosus, CDLE - Chronic discoid LE, Chron discoid lupus erythemat, lupus, lupus erythematosus discoid (diagnosis), lupus erythematosus discoid, discoid lupus erythematosus, Discoid lupus erythematosis, Lupus erythematosis discoid, Lupus erythematosus discoid, Lupus erythematosus discoides, Lupus Erythematosus, Discoid [Disease/Finding], dle, discoid lupus erythematosis, discoid lupus, discoid lupus erythematosus (DLE), lupus discoid, Chronic cutaneous lupus erythematosus, Discoid lupus, Discoid lupus erythematosus, DLE - Discoid lupus erythematosus, LE - Discoid lupus erythematosus, CDLE - Chronic discoid lupus erythematosus, Chronic discoid lupus erythematosus, Chronic discoid lupus erythematosus (disorder), Discoid lupus erythematosus (disorder), discoid; lupus, lupus; discoid, Discoid lupus erythematosus (disorder) [Ambiguous], Discoid Lupus Erythematosus, Lupus discoid, Discoid lupus erythematous
French LUPUS ERYTHEMATEUX DISCOIDE, LED, Lupus érythémateux chronique, Lupus érythémateux discoïde, Lupus érythémateux cutané chronique
Portuguese LUPUS ERITEMATOSO DISCOIDE, Lupus eritematoso discóide, Lúpus eritematoso discóide, Lúpus Eritematoso Cutâneo Crônico, Lúpus Eritematoso Discoide, Lúpus eritematoso cutâneo crónico, LUPUS ERITEMATOSO DISSEMINADO
Spanish LUPUS ERITEMATOSO DISCOIDE, Lupus eritematoso discoide, lupus eritematoso discoide crónico (trastorno), lupus eritematoso discoideo crónico (trastorno), lupus eritematoso discoide crónico, lupus eritematoso discoideo (trastorno), lupus eritematoso discoideo crónico, lupus eritematoso discoideo, Lupus eritematoso cutáneo crónico, LED, lupus eritematoso discoide (trastorno), lupus eritematoso discoideo (concepto no activo), lupus eritematoso discoide, Lupus Eritematoso Cutáneo Crónico, Lupus Eritematoso Discoide
Dutch discoïde lupus erythematodes, DLE, chronische cutane lupus erythematosus, discoïd; lupus, lupus; discoïd, Lupus erythematosus discoides, Lupus erythematosus, chronische cutane, Discoïde lupus erythematodes
German Lupus erythematosus diskoid, diskoider Lupus erythematosus, DLE, Lupus erythematosus diskoides, Lupus erythematodes discoides, Diskoider Lupus erythematodes, LE DISCOIDES, LE DISSEMINIERT, chronischer kutaner Lupus erythematodes, Lupus erythematodes, chronischer kutaner, Lupus erythematodes, diskoider, Lupus erythematodes, kutaner, chronischer
Italian Lupus eritematoso discoide (disseminato), Lupus eritematoso cronico cutaneo, Lupus eritematoso cutaneo cronico, Lupus eritematoso discoide
Swedish Lupus erythematosus, diskoid
Japanese エンバンジョウエリテマトーデス, DLE, DLE, エリテマトーデス-円板状, マンセイヒフエリテマトーデス, 円板状エリテマトーデス, 円板状紅斑性狼瘡, 慢性円板状エリテマトーデス, 慢性皮膚エリテマトーデス, 紅斑性狼瘡-円板状, エリテマトーデス-慢性皮膚, エリテマトーデス-皮膚-慢性, 慢性皮膚エリトマトーデス
Czech lupus erythematosus diskoidní, Diskoidní lupus erythematosus, Chronický kožní lupus erythematosus
Finnish Diskoidi lupus erythematosus
Korean 원판상 홍반성 루푸스
Polish Toczeń rumieniowaty ogniskowy, Liszaj rumieniowaty przewlekły
Hungarian Lupus erythematosus discoid, discoid lupus erythematosus, Discoid lupus erythematosus, Discoid lupus erythematosis, Discoid erythemás lupus, DLE, Chronikus cutan lupus erythematosus
Norwegian Lupus erythematosus discoides, Diskoid lupus erythematosus

Ontology: ale beer (C0678386)

Concepts Food (T168)
English ale beer, ales, ale