Lupus erythematosus subacute-cutaneous L93.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

annularly gyrated lupus erythematosus; Lupus erythematodes chronicus superficialis disseminatus; Lupus erythematosus anular gyrated; Lupus erythematosus drug-induced; SCLE; Subacute cutaneous lupus erythematosus

History
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Sontheimer, 1979

Definition
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Independent photosensitive (photosensitivity is expected in about 80% of patients - Black et al. 2002) subgroup of cutaneous lupus erythematosus with intermittent activity of disseminated, red, mostly blurred, unstructured (without detailed pattern) plaques, which occur preferentially in light-exposed skin areas. It is not uncommon to find anular detail patterns. 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 relation between the occurrence of the skin changes and the previous UV-exposure, which may have taken place months before). Higher risk of systemic involvement than with the chronic discoid form ( Lupus erythematodes chronicus discoides).

Etiopathogenesis
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Sun exposure. There is a varying degree of UV sensitivity.

In about 25-30% of cases, drugs are assumed to be the trigger. Possibly due to abnormalities in drug metabolism (polymorphism of hepatic acetyltransferase, slow acetylator) or they are drugs which per se have a photosensitizing effect.

The following drugs have been described in connection with the initiation or excision of SCLE: non-steroidal anti-inflammatory drugs (e.g. Piroxicam), tetracyclines, antimycotics ( griseofulvin; terbinafine), antihypertensives (captopril, cilazapril, hydrochlorothiazide), proton pump inhibitors.

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

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

Manifestation
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almost exclusively white women; first manifestation between 30-50 years.

Localization
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Areas exposed to light: face, with the centre of the face rather recessed, upper thorax, arms.

Clinical features
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Integument: Acute, chronically active, several or numerous, disseminated, sharp or blurred, 0.2-5.0 cm in diameter (rarely > 10.0 cm), homogeneous, anular or gyrated, red or reddish-brown spots or plaques with smooth or crusty surface.

Erythema exsudativum multiforme-like skin lesions are also possible.

Characteristic are (temporarily persistent) hypopigmentations (more rarely also hyperpigmentations) after healing of the lesions.

Extracutaneous manifestations: renal (16%), haematological pathologies (8%); slight visceral involvement with polyarthritis, serositis (1%), more rarely cerebrovascular diseases.

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

Laboratory
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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.

Histology
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Basically the same as in cutaneous lupus erythematosus, but less pronounced hyperkeratosis. Atrophic epithelium, orthokeratosis, vacuole degeneration of the basal epithelial cells, civatte bodies. Severe dermal edema with mucin deposits, dense, perivascularly oriented lymphocytic infiltrate.

Direct Immunofluorescence
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Negative in 40-50% of cases. Otherwise changes as in cutaneous lupus erythematosus (see below lupus erythematosus chronicus discoides).

External therapy
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External glucocorticoids (e.g. Dermatop) are only moderately successful. It is important to protect against sunlight in the UVB as well as in the UVA range!

Internal therapy
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  • 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 the clinic.
  • Chloroquine (e.g. Resochin): Start with 250 mg/day, after 2-4 weeks reduce to 250 mg every 2nd day. Cave! Ophthalmologic controls.

Progression/forecast
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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.

Literature
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  1. Amato L et al (2003) Subacute cutaneous lupus erythematosus in childhood. Pediatric dermatol 20: 31-34
  2. Black DR et al (2002) Frequency and severity of systemic disease in patients with subacute cutaneous lupus erythematosus. Arch Dermatol 138: 1175-1178
  3. Bottomley WW et al (1995) Methotrexate for the treatment of severe mucocutaneous lupus erythematosus. Br J Dermatol 133: 311-314
  4. Callen JD (2009) Clinically relevant information about cutaneous lupus erythematosus. Arch Dermatol 145: 316-319
  5. Farhi D et al (2006) Terbinafine - iduced subacute cutaneous lupus erythematosus. Dermatology 212: 59-64
  6. García AG et al (2014) Subacute cutaneous lupus erythematosus associated with pemetrexed plus carboplatin chemotherapy. J Clin Rheumatol 20:449-450

  7. Mutasim DF (2003) Severe subacute cutaneous lupus erythematosus presenting with generalized erythroderma and bullae. J Am Acad Dermatol 48: 947-949
  8. Pramatarov K et al (2000) Subacute cutaneous lupus erythematosus presenting with generalized poikiloderma. J Am Acad Dermatol 42: 286-288
  9. Sontheimer RD et al (1979) Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol 115: 1409-1415
  10. Wollenberg A et al (1991) Thiazide diuretic-induced subacute cutaneous lupus erythematosus. dermatologist 42: 709-712
  11. Tebbe B et al (1994) Course and prognosis of subacute cutaneous lupus erythematosus. A prospective study of 34 patients. dermatologist 45: 690-695
  12. Wollina U et al (1989) On the spectrum of dermatological manifestations in patients with subacute cutaneous lupus erythematosus and their nosological significance. Act Dermatol 15: 208-212

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Last updated on: 29.10.2020