Chronic discoid lupus erythematosus (CDLE)

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: EB51.0

Cazenave (Paris), 1844

Integumentary lupus erythematosus, lupus erythematosus chronicus discoides, lupus erythematosus chronicus, discoid lupus erythematosus (DLE), discoid lupus erythematosus.

Chronic cutaneous form of lupus erythematodes (LE).

  • Most common cutaneous form of LE
  • First manifestation is usually between 20-40 yrs
  • Females are more commonly affected: 2:1-4:1
  • More common in dark-skinned individuals

  • Division
    • Localised form (approx. 80 %): head
    • Disseminated form (approx. 20 %): head, trunk and extensor sides of extremities
    • DLE of the oral mucosa: especially buccal mucosa is affected, less frequently the palate
  • Genetics almost certainly play a role
  • Trigger factors
    • Cold.
    • UV radiation (triggering reproducible in 30-40% of patients by light (UVB))
      • Tanning beds can also trigger an exacerbation, and in rare cases lupus nephritis

  • Discoid, sharply demarcated, erythematous plaques with partial follicular hyperkeratosis. Some patients report hyperaesthesia in the area of the skin lesions. Dolent "tape nail phenomenon". The skin lesions heal leaving atrophic skin
  • There are few plaques, some occurring solitarily
  • Pigmentary shifts (hypo/hyperpigmentation), telangiectasias note in the centre of the foci
  • The palpation is coarse/hardened, on the other hand hardly raised above the surrounding skin surface
  • Healing at the capillitium may occur in the setting of scarring alopecia. Scarring periorale, in the nasal and oral mucosa

  • Clinical findings
  • Biopsy with direct immunofluorescence
  • Autoantibodies are mostly negative
    • ANA (5% ↑), usually.
    • Anti-dsDNA (rare).
    • Ro/SSA (rare).
    • U1-RNP (rare).

UV-exposed areas.

Moderate compact ortho- and parahyperkeratosis, follicular hyperkeratosis, acanthosis or atrophy of the epidermis, hydropic degeneration of the cells of the stratum basale, necrotic/apoptotic keratinocytes in the basal epidermis (colloid bodies), oedema in the upper corium, interfaced dermatitis, widened PAS-positive basement membrane, periadnexal and perivascular lymphocytic infiltrates.

Direct immunofluorescence: In affected, but not in unaffected skin, a positive lupus band test with IgG, IgM, IgA, C1 and C3 is found.

The disseminated form more often progresses to SLE than the localised form.

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