Lupus erythematosus, cutaneous type

Special form of lupus erythematosus, which is only localised on the skin. Internal organs are not affected

• 10x more patients develop cutaneous lupus erythematosus than systemic lupus erythematosus (SLE)
• Peak age of first manifestation: 40-60 yrs.

• Cutaneous lupus erythematosus (CLE)
  • Acutaneous cutaneous lupus erythematosus (ACLE)
  • Localised ACLE.
  • Generalised ACLE
    • Subacute cutaneous lupus erythematosus (SCLE)
      • Annular SCLE.
      • Papulosquamous SCLE.
• Intermittent cutaneous LE (ICLE)
• Chronic cutaneous lupus erythematosus (CCLE)
  • Discoid lupus erythematosus
    • Localised DLE
      • Lupus erythematosus verrucosus (hypertrophicus)
      • Lupus erythematosus tumidus
      • Lupus erythematosus profundus (lupus erythematosus panniculitis)
      • Mucosal DLE
      • Oral DLE
      • Conjunctival DLE
    • Generalised DLE
• Bullous CLE
• Special forms
  • Rowell's syndrome
  • Chilblain lupus

Possible triggers and trigger factors:

• UV light
• Medicines
• Genetics (HLA association)
• Smoking
• Köbner phenomenon
• Estrogens (gravidity, ovulation inhibitors)
• Vaccinations
• Neoplasms

• Anamnesis
• Clinic
• Biopsy
• Note: In individual cases, direct immunofluorescence (DIF) from an affected skin area that is preferably not exposed to light can be helpful
• If necessary, photoprovocation testing
  • To objectify the individual photosensitivity of a patient
  • Indication:
    • If diagnosis is uncertain or
    • the photosensitivity cannot be ascertained with certainty from anamnesis
• Serology:
• The AWMF guideline on cutaneous lupus erythematosus recommends:
• When cutaneous lupus erythematosus is suspected:
  • BB (anemia in 0-27%, leukocytopenia in 0-30%, thrombocytopenia in 2-4%), ESR, C-reactive protein (CRP), GOT, GPT, γGT, AP, LDH, CK, serum creatinine, urine sediment, ANA titer and pattern.
• If ↑ serum creatinine:
  • Creatinine clearance in 24-h collection urine
  • Calculation of glomerular filtration rate according to MDRD formula
• Determination of cystatin C.

After dermatopathological confirmation of the diagnosis:

• In case of pos. ANAs: anti-dsDNA and ENAs
• C3, C4
• Antiphospholipid antibodies, PTT
• Immunoglobulins
• Rh factor
• TSH, thyroid antibodies:
  • MAK (microsomal antibodies) or TPO antibodies (thyroperoxidase antibodies)
• TAK (thyroglobulin antibodies)
• TRAK (TSH receptor antibody)
• VDRL.
• 24 hour urine (protein and creatinine)
• Glucose-6-phosphate dehydrogenase activity

Source: Kuhn, A. (2016). S1 guideline: Cutaneous Lupus Erythematosus. Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/013-060.html

• Apparative diagnostics (according to AWMF guideline "Cutaneous lupus erythematosus")
• ECG
• Echocardiography
• Pulmonary function with measurement of CO diffusion capacity
• Thoracic X-ray
• Upper abdominal sonography
• If necessary, rheumatological presentation with ultrasound, duplex sonography and, if necessary, X-ray of the joints

Source: Kuhn, A. (2016). S1 guideline: Cutaneous Lupus Erythematosus. Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/013-060.html

Notice!

• To evaluate the effectiveness of therapy for cutaneous lupus erythematosus in trials, the use of the CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index) is recommended.

• Face frequently, sun-exposed areas
• Ears less frequent, but typical localisation

Sun exposure and deterioration afterwards?

S. spec. diseases.

• Mutilations due to scarring
• Spinocellular carcinoma
• Alopecia

• Chronic course
• Transition to systemic lupus erythematosus (in approx. 5%)

Avoid common mistakes:

• History should always include photosensitivity
• Medication history to rule out "drug-induced" lupus erythematosus
• Family and self history for autoimmune diseases
• Do not overestimate the ARA criteria! The ARA criteria are only conditionally suitable for the exclusion of systemic lupus erythematosus
• Avoid a purely superficial biopsy
• Therapy should not be changed until preventive measures to avoid possible trigger factors have been exhausted and sunscreen with a high sun protection factor is applied daily!

Standard Dermatology USB

General measures

• Discontinuation of possible triggering drugs
• Avoid UV radiation
• Apply sunscreen for UVA and UVB (SPF ≥ 50)
several times a day
• If necessary, UV protection films for windows
• Textile UV protection (hats and clothing)
• Camouflage cosmetics
• Avoid taking photosensitising medicines

Topical therapies

1. Choice:

• Glucocorticosteroids
  • Topical e.g.:
    • Elocom® cream 1x daily in tapering application frequency on the body. If necessary, short-term occlusive application.
    • Alfacorton® cream 1x daily in creeping application frequency on the face.
    • Intralesional application with e.g..: Kenacort® 40 mg/ml, CAVE risk of atrophy
  • Calcineurin inhibitors (off-label)
    • Tacrolimus ointment 0.1% 1-2x tgl
    • Pimecrolimus cream 1% 1-2x tgl.
• Other alternatives
  • Retinoids (off-label)
    • Adapalene 1x tgl. less often if necessary
• Laser (argon, pulsed dye, CO2 laser)

Systemic therapy

Therapy of 1st choice according to AWMF guideline

• Hydroxychloroquine p.o. 6.0 - 6.5 mg/kg bw/d, off-label for cutaneous lupus erythematosus (approved for SLE)
• Chloroquine p.o. 3.5 - 4.0 mg/kg bw/d in cutaneous lupus erythematosus off-label (approved for SLE)
• Mepacrine p.o. 100 mg 1x tgl
  • In case of therapy resistance, if necessary in combination with chloroquine or hydroxychloroquine
  • Off-label therapy
• Glucocorticosteroids e.g. prednisolone <0.5 - 1.0 mg/kg bw/d
  • Indication: in acute cases, to bridge the gap until other medication takes effect, with pronounced symptoms
  • Off-label therapy

Therapy of 2nd choice according to AWMF guideline

• Methotrexate 7.5 - 25 mg (target: 0.2 mg/kg bw) p.o., s.c., i.v., i.m. (s.c. recommended at the USZ) 1x a week
  • Off-label therapy
  • Effect: SCLE > DLE, localised DLE > disseminated DLE
  • Off-label therapy
• Retinoids:
  • Acitretin p.o
    • Initial:0.5 mg/kg bw 1x tgl.
    • Maintenance dose: 0.1-0.2 mg/kg bw 1x tgl.
    • Blood samples: Before therapy and every 8 weeks: GOT, GPT, gamma-GT, triglycerides, cholesterol, creatinine, HDL; in women, reliable conception protection and monthly pregnancy tests
    • KI: severe renal or hepatic insufficiency, hyperlipidaemia
    • WW: with vitamin A (retinoids), tetracyclines and methotrexate; no blood donation (up to 1 year after therapy), pregnancy (up to 2 yrs after therapy), lactation
    • Indication: Hypertrophic DLE, possibly SCLE, CLE/ichen planus overlap
    • Off-label therapy
  • Isotretinoin p.o. 0.5 - 1.0 mg/kg bw/d
    • Indication: hypertrophic DLE, possibly SCLE, CLE/ichen planus overlap
    • Off-label therapy
  • Dapsone p.o. 50 - 200 mg/d
    • Approved for bullous, ulcerative or urticarial lupus erythematosus
    • Indication: localised DLE, oral ulcers, urticarial vasculitis, LEP, SCLE.
  • Mycophenolate mofetil (MMF) p.o. 2 x 1000 mg respectively
  • Mycophenolatsodium (MP-S) p.o. 2 x 720 mg respectively
  • Indication: mainly SCLE, or also CHLE.
  • Off-label therapy

Therapy of 3rd choice according to AWMF guideline

• Thalidomide p.o. 50-200 mg/d
  • Indication: DLE, LEP and SCLE.
  • CAVE: polyneuropathy
  • Off-label therapy
• IVIG 1-2 g/kg bw every 4 weeks
  • Severe refractory CLE
  • CAVE: ↑ Cost
  • Off-label therapy
• Clofazimine 100 - 200 mg/day
  • Indication: severe refractory DLE, also other CLE forms
  • Off-label therapy
• Phenytoin 3 x 100 mg/d
  • Indication: severe refractory CLE.
  • Off-label therapy

Not recommended is the use of azathioprine.

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