Chronic discoid lupus erythematosus (CDLE)

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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