Lupus erythematosus subacute-cutaneous L93.1

Sontheimer, 1979

Independent photosensitive (photosensitivity is expected in about 80% of pat - Black et al. 2002) subgroup of cutaneous lupus erythematosus with relapsing activity of disseminated, red, usually blurred structureless (without detail pattern) plaques, which occur preferentially in light-exposed skin areas. Not infrequently, anular detail patterns are also found. The light-relatedness results from the macro-pattern, which more often (not always) omits non-UV-exposed skin areas (note: there is often no temporal relationship between the appearance of the skin changes and the previous UV exposure, which may have occurred months before). Higher risk of systemic involvement than in the chronic discoid variant(lupus erythematosus chronicus discoides).

Sun exposure. There is varying degrees of UV sensitivity.

Drugs are thought to be the trigger in about 25-30%. Possibly due to abnormalities in drug metabolism (polymorphism of hepatic acetyltransferase, slow acetylators) or they are drugs that are photosensitizing per se.

The following drugs have been described in association with the initiation or exacerbation of SCLE: nonsteroidal anti-inflammatory drugs (e.g., piroxicam), tetracyclines, antifungals(griseofulvin; terbinafine), antihypertensives (captopril, cilazapril, hydrochlorothiazide), proton pump inhibitors.

Also described is occurrence after ingestion of spironolactone, cinnarizine, D-penicillamine, interferon beta, ranitidine, antidiabetics(sulfonylureas), doxorubicin, docetaxel, carboplatin (see below cytostatics, extravasates).

Pathogenetically relevant appears an increased susceptibility in the presence of HLA types B8, DR2, DR3, DQw2, DRw52.

Predominantly fair-skinned / Caucasian women; first manifestation between 30-50 years of age.

Integument: Acute, chronically active, multiple or numerous, disseminated, sharply or also indistinctly circumscribed, 0.2-5.0 cm in size (rarely > 10.0 cm), disc-shaped, anular or gyrated, red also red-brown spots or plaques with a smooth but also crusty surface.

Erythema multiforme-like skin lesions are also possible.

Characteristic are (temporarily persisting) hypopigmentation (more rarely also hyperpigmentation) after healing of the lesions.

Extracutaneous manifestations:

renal (16%)

hematologic pathology (8%)

minor visceral involvement with polyarthritis, serositis (1%)

less frequent are cerebrovascular erscehinungen.

The clinical picture of drug-induced SCLE is identical, but mostly in the form of disseminated exanthema. The triggers are mainly hydrochlorothiazide, terbinafine, proton pump inhibitors.

At 75% higher titre ANA, more rarely anti-DNA antibodies. Particularly characteristic is the detection of anti-SSA/Ro-antibody (about 50% of cases) and anti-SSB/La-antibody. Possibly leukopenia (cytopenia), hypergammaglobulinemia.

External glucocorticoids (e.g. Dermatop) are only moderately successful. Light protection is important - both in the UVB and UVA range!

Initial glucocorticoids such as prednisone (e.g. Decortin) 20-40 mg/day p.o., possibly higher doses. Alternatively cloprednol (Syntestan) 10-15 mg/day. Reduce dosage depending on clinic.

Chloroquine (e.g., Resochin): Start with 250 mg/day, after 2-4 weeks reduce to 250 mg every 2nd day. Caveat. Ophthalmologic controls.

Quoad vitam cheap. Chronic course is characteristic.

Skin alterations usually heal completely. Hypo- or hyperpimplantations are possible.

In more than 50% of patients more than 4 ARA criteria (see lupus erythematosus, systemic) are positive.

About 15% of patients develop internal involvement (arthralgias, nephritis, cardiac involvement, hematological complications) during the course of their disease and meet the criteria of systemic lupus erythematosus.

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