Chronic discoid lupus erythematosus (CDLE)
Last Updated: 2022-03-11
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
- 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
- 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).
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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