Rheumatology Book

Connective Tissue Disorders

  • Cutaneous Lupus Erythematosus

Intra-Articular Disorders

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Cutaneous Lupus ErythematosusAka: Cutaneous Lupus, CLE, CCLE, SCLE, ALE, Discoid Lupus Erythematosus, Discoid Lupus, Chronic Cutaneous Lupus, Scarring Discoid Lupus, Subacute Cutaneous Lupus, Acute Cutaneous Lupus

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  1. See Also
    1. Systemic Lupus Erythematosus (SLE)
  2. Classification
    1. Chronic Cutaneous Lupus (CCLE) or discoid lupus (DLE)
      1. Peak Incidence in ages 30 to 40 years
      2. More common in women
      3. Exacerbated by trauma and sunlight
      4. Asymmetric red 1-2 cm Plaques with adherent scale
        1. Progresses to local skin atrophy over months
        2. Ultimately results in scar
          1. Scar with altered pigmentation
          2. Usually depressed, but may be hypertrophic
    2. Subacute Cutaneous Lupus (SCLE)
      1. Mid-category between CCLE and ALE
      2. Usually heals without scarring (unlike CCLE)
      3. Annular Lesion
        1. Usually involves mid-trunk
        2. Central Hypopigmentation and telangiectasia
    3. Acute Cutaneous Lupus (ALE)
      1. Red Plaques
        1. Occurs on face, chest, and shoulders
        2. Extensor arm and hand dorsum also involved
      2. Malar Rash may also occur
  3. 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 (ALE): 98%
      2. Anti-dsDNA
        1. Subacute Cutaneous Lupus (SCLE): 30%
        2. Acute Cutaneous Lupus (ALE): 60-80%
      3. Complement (C3 or CH50)
        1. Suppressed in 90% of Acute Cutaneous Lupus (ALE)
    3. Further evaluation
      1. See Systemic Lupus Erythematosus
      2. Distinguish systemic from isolated skin involvement
  4. Management
    1. Sunscreen with UVA and UVB protection is critical
    2. Topical Corticosteroids (first choice)
    3. Intralesional Corticosteroid
      1. Indicated for Discoid lupus (DLE, CCLE)
    4. 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
  5. References
    1. Habif (1996) Dermatology, p. 544-9
    2. Patel (2002) Dermatol Clin 20(3):373

Barrett Esophagus (C0004763)

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 (NCI)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 segment 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, 2000) -- 2003
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.
ConceptsDisease or Syndrome (T047)
ICD9530.85
MSHD001471
EnglishBarrett Esophagus, Barrett Syndrome, Barrett's Esophagitis, Barrett's Esophagus, Barrett's esophagus with esophagitis, Barrett's oesophagus, Barrett's oesophagus with oesophagitis, Barrett's syndrome, Barrett's ulcer, Barrett's ulcer of esophagus, Barrett's ulcer of oesophagus, Barretts esophagus, Barretts Syndrome, BE, BO - Barrett's esophagus, BO - Barrett's oesophagus, CELLO, CELLO - Columnar epithelial-lined lower esophagus, CELLO - Columnar epithelial-lined lower oesophagus, CLE, CLE - Columnar-lined esophagus, CLE - Columnar-lined oesophagus, Columnar epithelial-lined lower esophagus, Columnar epithelial-lined lower oesophagus, Columnar-lined esophagus, Columnar-lined oesophagus, Endobrachyoesophagus, Gastric metaplasia of esophagus, Gastric metaplasia of oesophagus
FrenchEndobrachyoesophage, Oesophage de Barrett
ItalianEndobrachiesofago, Esofago di Barrett
Spanishepitelio columnar en el esofago distal, esofago con epitelio columnar, esofago de Barret, esofago de Barret con esofagitis, metaplasia gastrica de esofago, metaplasia gastrica del esofago, sindrome de Barrett, ulcera de Barret, ulcera esofagica de Barret
Parent ConceptsEsophageal Diseases (C0014852), Syndrome (C0039082), [X]Other specified diseases of esophagus (C0348727), Congenital digestive system anomalies (C0266015), Esophageal Precancerous Condition (C1333465), Precancerous Conditions (C0032927), Barrett Esophagus (C0004763), Esophagitis (C0014868), Ulcer of esophagus (C0151970), Soft tissue lesion (C0410013), Ambiguous concept (C1274012), Disorder of gastrointestinal tract mucous membrane (C1290615), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, DXP, ICD9CM, MEDLINEPLUS, MSH, MTH, MTHMST, MTHMSTFRE, MTHMSTITA, NCI, NDFRT, OMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


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.
ConceptsDisease or Syndrome (T047)
ICD9695.4
MSHD008179
EnglishCDLE - Chronic discoid lupus erythematosus, Chronic discoid lupus erythematosus, DISCOID LUPUS, DISCOID LUPUS ERYTHEMATOSIS, Discoid lupus erythematosus, DLE, DLE - Discoid lupus erythematosus, LE - Discoid lupus erythematosus, lupus, Lupus discoid, LUPUS ERYTHEMATOSIS DISCOID
SpanishLED, lupus eritematoso discoide, lupus eritematoso discoideo, lupus eritematoso discoideo cronico
Parent ConceptsSkin finding (C0455205), skin disorder (C0037274), Exanthema (C0015230), Erythema (C0041834), COLLAGEN VASCULAR DISEASE (C0262428), Other skin disorders (C0549660), Lupus Erythematosus, Cutaneous (C0024137), Lupus Erythematosus, Discoid (C0024138), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesAIR, COSTAR, CSP, CST, DXP, MSH, MTH, MTHICD9, NCI, NDFRT, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


ale (C0678386)

ConceptsFood (T168)
Englishale
Parent Conceptsspecific beer types (C0678385)
SourcesAOD
Derived from the NIH UMLS (Unified Medical Language System)



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