Lupus erythematodes chronicus discoides L93.0

Most frequent form of cutaneous lupus erythematosus with circular, oval or garland-shaped, red plaques with flat, lesional, mostly small-spotted adherent scaling (which may also be absent). Characteristic follicular hyperkeratosis can occur when scaling is present. Typical, and thus diagnostically groundbreaking for CDLE, is scarring, which usually occurs in the centre of the lesion. In older lesions the circumscribed scarring may dominate the clinical picture. In older lesions, the scarring can dominate the clinical picture, and red or whitish, scaly plaques can cover a light scar area. The healed lesion of chronic discoid lupus erythematosus is a non-irritant, sunken scar. The process of scarring healing can take years.

Hypertrophic chronic discoid lupus erythematosus ( lupus erythematosus verrucosus) is to be regarded as a variant.

Incidence: 15-50/100.000 inhabitants/year. Worldwide occurrence. African Americans have a higher prevalence than Caucasian Americans. Asians show similar prevalence to Caucasians.

Unknown. Autoimmune disease with genetic predisposition. Provocation and entertainment of manifestation by exogenous factors such as trauma, light, cold, stress, infections.

Mainly occurring in adults (20 to 40 years of age).

f:m=3:1

Rarely heredity exists.

Mostly face (cheeks, forehead, nose), auricles, but also hairy head, chest, rarely trunk and extremities.

Sharply demarcated, 1.0-10.0 cm large, solitary, few or multiple, homogeneously reddened, scaly, usually only slightly elevated, but clearly indurated red plaques. Peripheral growth may result in circular, sometimes annular, structures with a flaking scarred center(atrophy of follicular ostia; look for it carefully!) and peripheral zone of progression. Possibly confluence of the foci. Typical is a hypersensitivity of the inflammatory zone (recommendation: brush with light spatula pressure from the healthy skin into the diseased skin). In the scarring center of the discoid foci, depigmentation, hyperpigmentation and/or telangiectasia are also seen. Solitary or multiple, hypersensitive foci of alopecia with central scarring and, depending on the activity, peripheral inflammatory progression zone appear at the capillitium. S.a.u:

• Alopecia, scarring
• Lupus erythematosus tumidus
• Chilblain's lupus
• Lupus erythematosus profundus
• Lupus erythematosus verrucosus.

Characteristic of discoid lupus erythematosus on reflected light microscopy are follicular hyperkeratosis in the foci, and hyperesthesia when the foci are touched(tapestry nail phenomenon).

Mostly without pathological findings. Rarely leukopenia and positive (low-titer) antinuclear antibodies (ANA). It is important to exclude SLE (see below lupus erythematosus, systemic).

In affected skin detection of IgG, IgM, IgA,_C1 and_C3 at the dermo-epidermal junction zone (= positive lupus band test); unaffected skin is negative.

Exclusively external measures are not always sufficient!

• Consistent light protection. Use of sunscreens with a high protection factor, UVA and B filters (preferably sun protection preparations on a physical basis) and consistent textile sun protection. It is important to inform the patient comprehensively about the various sun protection measures.
• Topical glucocorticoids. Depending on the localisation and activity of the HV, an appropriate class of active substances is selected. Possibly occlusive dressings.
• Capillitium: Steroid-containing solution. (e.g. Ecural-Lsg.) once/day, if necessary additional injection with glucocorticoid crystal suspension( triamcinolone acetonide, e.g. Volon A, 10-20 mg together with 2 ml 1% mepivacaine and applied several times with a thin cannula at intervals of 4-6 weeks intralesionally).
• Topical immunomodulators: In pilot studies good effects by local treatment with tacrolimus (e.g. 1% protopic ointment) or Pimecrolimus (e.g. Elidel).

Antimalarials: Hydroxychloroquine (^Quensyl®) or chloroquine (^Resochin®) are the first choice! They show satisfactory results in 50-90% of patients. Dosage of chloroquine: initially 4.0 mg/kg bw/day; children: 3.5 mg/kg bw/day. After one month disease-adapted reduction of the dose. Regular ophthalmologic check-ups due to possible retinopathy. Chloroquine leads to a reduction of HLA-DR+/CD1a+ cells in lesional skin.

Alternative: hydroxychloroquine (^Quensyl®, dosage: 6.5 mg/kg bw/day; children: 6.0 mg/kg bw/day), disease-adapted reduction of the dose. Total dose 100 mg. In individual cases with good tolerance, the total dose can be significantly exceeded.

Please note! Smokers respond significantly less well to treatment with antimalarials than non-smokers! Pregnancy with antimalarials is not a contraindication. Note: Antimalarials such as cloroquine and hydroxychlorquine can trigger hemolytic crises in the presence of glucose-6-phosphate dehydrogenase deficiency. In this respect, the patient should be asked about this (FA; signs of favism).

If necessary, combine with glucocorticoids (e.g. prednisolone 5 mg/day p.o.).

In case of extensive findings and lack of response to other therapies glucocorticoid shock therapy: Initially 100 mg/day prednisolone (e.g. Solu-Decortin H) i.v.; slowly taper off. Maintenance dose: Prednisolone 10-20 mg/day (e.g. ^Decortin® H) p.o. If necessary, combination with azathioprine (e.g. Imurek ^®50-100mg/day) p.o.

Retinoids such as acitretin (neotigason 10-20 mg/day) are used as an alternative to chloroquine. The therapeutic successes are not particularly convincing.

Studies with methotrexate (intravenous therapy at 15-25 mg/week) and mycophenolate mofetil (MMF) show good clinical success. MMF is used in particular in patients who are refractory to antimalarials. MMF can be used alone or in combination with antimalarials in a dosage of between 1500 and 3500 mg p.o./day.

Thalidomide: can be used as an alternative in severe cases of CDLE (Chasset et al. 2018). A high response rate (90%) is offset by high rates of treatment discontinuation (24%) due to side effects (neuropathy, thromboembolic processes).

• Physical or surgical measures should be considered after weighing the risk-benefit ratio against other procedures. To be considered:
• Cryosurgery in open or closed procedures; for stamping procedures, cool the stamp surface to 0 °C, place the stamp loosely and freeze briefly until the stamp temperature (not tissue temperature) of -150 °C is reached. Repeat the procedure immediately after thawing.
• Cauterization: Lesions can also be carefully coagulated with electrocautery; surface scabbed, post-cure with sharp curette (boot curette no. 6).

Chronic course, acute exacerbation with visceral involvement possible; quod vitam favourable, defect healing. A transition to systemic lupus erythematosus is reported in about 1% (other sources say 5-10%!!) of cases.

Many patients report light sensitivity, possibly burning and itching after UV exposure.

However, a smaller number of patients seem to benefit from sun exposure.

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