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derm.cutaneous-lupus.core.v1PRODUCTION
derm.cutaneous-lupus.core.v1

Cutaneous lupus erythematosus (ACLE / SCLE / discoid + variants)

dermatologychronicsubacuteadult
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Frame as a CHRONIC/subacute photosensitive autoimmune skin-disease spectrum (ACLE strongly SLE-associated; SCLE anti-Ro + drug-induced subset; DLE scarring/irreversible) managed on a photoprotection-first stepwise ladder. Core tasks: confirm CLE (CLASI activity vs damage), screen EVERY patient for systemic lupus (ACLE/SCLE high-risk), enforce photoprotection + smoking cessation (antimalarial efficacy), and reconcile drugs (drug-induced SCLE). The not-to-miss is active SYSTEMIC lupus hiding behind the rash.

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CLE-spectrum framing set; SLE-screen + drug-induced-SCLE + scarring escape routes noted

Patient inputs (16)

Photodistribution + UV provocation supports CLE vs the non-photosensitive mimics (rosacea, lichen planus) and anchors the photoprotection mandate (German S2k Part 1 PMID 34390136)

EVERY CLE patient is screened for SLE — arthralgia, serositis, nephritis, cytopenia, photoworsened systemic flare; ACLE/SCLE high-risk, DLE lower; positive screen routes to rheum.sle.core.v1 (Lu et al CLE guideline PMID 34364171)

SCLE has a major drug-induced subset (PPIs, thiazides/ACE-i/CCB, terbinafine, anti-TNF, taxanes, chemotherapeutics) — reconcile and withdraw the culprit; resolution on withdrawal is the pivot (He & Sawalha PMID 29870500)

Smoking blunts antimalarial (hydroxychloroquine) efficacy in CLE — cessation is a core treatment lever, captured before the systemic ladder (Ezra & Jorizzo PMID 22582908; Dutz & Werth PMID 21918570)

True treatment failure (adequate potency/photoprotection/antimalarial dose/duration/adherence + smoking-cessation) vs under-treatment determines whether to escalate or optimise (Lu et al CLE guideline PMID 34364171)

Malar ACLE SPARES the nasolabial folds and lacks pustules/telangiectasia; rosacea is centrofacial with the folds involved — the highest-yield ACLE-vs-rosacea pivot (route derm.rosacea.core.v1) (German S2k Part 1 PMID 34390136)

ACLE (malar, transient, sparing nasolabial folds) vs SCLE (annular/papulosquamous, non-scarring) vs DLE (fixed, scarring, follicular plugging, dyspigmentation) is the diagnostic pivot and drives SLE-risk and scarring-urgency (Lu et al CLE guideline PMID 34364171)

Anti-Ro/SSA (SCLE-associated) in a pregnant/conceiving patient → neonatal-lupus / congenital-heart-block risk → fetal cardiology + pregnancy-compatible regimen (continue HCQ, avoid teratogens) (German S2k Part 2 PMID 34338428)

DLE scarring + dyspigmentation + scarring (cicatricial) scalp alopecia is irreversible — extent/activity drives early aggressive therapy and the damage-vs-activity (CLASI) distinction (German S2k Part 2 PMID 34338428)

Antimalarials are usable in pediatric/pregnant CLE per guideline; thalidomide/lenalidomide neuropathy risk and dosing differ by age/comorbidity (Lu et al CLE guideline PMID 34364171)

Lesional skin biopsy + direct immunofluorescence (lupus band — granular IgG/C3 at the dermo-epidermal junction) confirms interface dermatitis and discriminates the mimics (German S2k Part 1 PMID 34390136)

ANA + anti-Ro/SSA (SCLE) + anti-dsDNA/anti-Sm (SLE-specific) stratify subtype and systemic risk; drug-induced SCLE is typically anti-Ro positive (German S2k Part 1 PMID 34390136)

SLE haematologic surveillance (cytopenias) + thalidomide/MMF/MTX/azathioprine myelosuppression baseline + monitoring (Lu et al CLE guideline PMID 34364171)

Lupus-nephritis surveillance (creatinine, urinalysis/protein) — a positive renal screen converts CLE management to systemic-lupus pathway; CKD-EPI 2021 race-free eGFR for renal dose-adjust (Cochrane PMID 33687069; Inker NEJM 2021)

Methotrexate hepatotoxicity + mycophenolate baseline + on-treatment monitoring in the refractory ladder (Lu et al CLE guideline PMID 34364171)

Thalidomide/lenalidomide (REMS), mycophenolate, methotrexate are teratogenic and contraindicated; hydroxychloroquine is continued in pregnancy; gates the ladder (Lu et al CLE guideline PMID 34364171)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (7)

7 need judgement
  • informationalseverecle_with_active_systemic_lupus_organ_involvement
    CLE rash accompanied by active SLE organ involvement — lupus nephritis, cytopenia, serositis, or systemic flare (esp. ACLE/SCLE)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererapidly_scarring_dle_or_scalp_scarring_alopecia
    Active discoid LE with rapidly progressive scarring / dyspigmentation, or expanding scalp cicatricial (scarring) alopecia
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverescle_in_pregnancy_with_anti_ro_ssa
    SCLE (or any CLE) in a pregnant/conceiving patient with anti-Ro/SSA positivity
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatedrug_induced_subacute_cle
    Annular/papulosquamous photodistributed SCLE temporally linked to a culprit drug (PPI, thiazide/ACE-i/CCB, terbinafine, anti-TNF, taxane, chemotherapeutic)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatehydroxychloroquine_retinopathy_signal
    Screening or symptomatic signal of antimalarial retinopathy (visual-field/SD-OCT/mfERG abnormality) or cumulative-dose / >5 mg/kg/day exposure
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatesevere_refractory_widespread_cle
    Widespread CLE refractory to adequate photoprotection + antimalarial (≥3 mo at target dose, smoking addressed), high CLASI activity
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildantimalarial_efficacy_undermined_by_smoking
    Active CLE in a current smoker on (or about to start) antimalarial therapy
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

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TREATMENToptionalDrives dose adjustment
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Recommended regimen

Cutaneous lupus erythematosus — photoprotection-first + stepwise ladder (Lu et al CLE guideline 2021; German S2k 2021)
axis: cle_photoprotection_first_stepwise_ladderstep 1 - Step 1 — Universal photoprotection + smoking cessation + vitamin D (every patient, every step, lifelong)
Selected step "Step 1 — Universal photoprotection + smoking cessation + vitamin D (every patient, every step, lifelong)" — All CLE subtypes/severities, continuously — the non-negotiable substrate; UV is the principal disease driver and smoking blunts antimalarial efficacy
  • rigorous_broad_spectrum_photoprotection
    first line
    photoprotection
    German S2k Part 2 (PMID 34338428) — broad-spectrum high-SPF (≥50) UVA+UVB sunscreen + photoprotective clothing + behavioural sun avoidance; UV is the principal CLE trigger and photoprotection prevents lesion induction.
  • smoking_cessation
    first line
    risk_factor_modification
    Ezra & Jorizzo (PMID 22582908) + Dutz & Werth (PMID 21918570) — smoking blunts antimalarial response in CLE; cessation is central to maximising hydroxychloroquine efficacy and long-term remission.
  • vitamin_d_repletion
    add on
    supplement
    triggers: photoprotection_associated_vitamin_d_insufficiency
    German S2k Part 2 (PMID 34338428) — rigorous photoprotection predisposes to vitamin-D insufficiency; supplement to maintain adequacy.

outpatient playbook — drug actions (4)

  1. 1. broad-spectrum photoprotection + smoking cessation + vitamin D (foundation)
    SPF≥50 UVA/UVB • topical/behavioural • daily, lifelong
    trigger: All CLE patients, all subtypes/severities (German S2k Part 2 PMID 34338428; Ezra & Jorizzo PMID 22582908)
    UV is the principal disease driver; smoking blunts antimalarial response
  2. 2. clobetasol 0.05% / intralesional triamcinolone / tacrolimus 0.1% (face)
    rxcui 21245
    0.05% / 2.5–10 mg/mL / 0.1% • topical/intralesional • pulsed / q4–6wk / BID
    trigger: Localised low-activity CLE (Lu et al CLE guideline PMID 34364171)
    Topical/intralesional corticosteroid + topical calcineurin first-line for localised disease; TCI steroid-sparing on face
  3. 3. hydroxychloroquine ≤5 mg/kg/day ± quinacrine add-on
    rxcui 5521
    ≤5 mg/kg/day • PO • once daily
    trigger: Widespread/severe or topical-refractory CLE; first-line systemic for ALL subtypes (Lu et al CLE guideline PMID 34364171)
    Antimalarial first-line systemic; baseline+annual ophthalmology; smoking-cessation maximises response
  4. 4. methotrexate / MMF / thalidomide (refractory DLE) / belimumab / anifrolumab; withdraw culprit if drug-induced SCLE
    rxcui 6851
    agent-specific • PO/SC/IV • agent-specific
    trigger: Antimalarial-refractory CLE or widespread CLE with active SLE (Lu et al CLE guideline PMID 34364171)
    Second/third-line immunosuppression; belimumab/anifrolumab for SLE-active widespread CLE; drug-induced SCLE → withdraw culprit

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Photodistributed erythematous eruption — malar/butterfly (ACLE), annular/papulosquamous (SCLE), or fixed scarring discoid plaques (DLE) (Lu et al CLE guideline PMID 34364171; German S2k Part 1 PMID 34390136); Atrophic scarring / dyspigmented plaque with follicular plugging ± scalp scarring (cicatricial) alopecia → discoid LE — IRREVERSIBLE damage, early aggressive Rx (German S2k Part 2 PMID 34338428); UV-provoked or photoaggravated rash (sun-exposure/seasonal pattern) — the CLE photosensitive entry (German S2k Part 1 PMID 34390136).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Cutaneous lupus erythematosus (ACLE / SCLE / discoid + variants)** (derm.cutaneous-lupus.core.v1).
Phenotype framing: Terminal photodistributed differential with named pivots: CLE (interface dermatitis + lupus band + photodistribution pivot) vs rosacea (centrofacial, INVOLVES nasolabial folds, pustules/telangiectasia, no scarring — route derm.rosacea.core.v1) vs psoriasis / papulosquamous SCLE (sharp salmon plaque + silver scale + nail pits — route derm.psoriasis.core.v1) vs polymorphous light eruption (recurrent monomorphic post-UV, self-limited, no interface/serology) vs dermatomyositis (heliotrope + Gottron + proximal myopathy + anti-Mi2/MDA5 — distinct) vs lichen planus (Wickham striae, oral/scalp interface overlap — route derm.lichen-planus.core.v1) vs cutaneous sarcoidosis (non-caseating granulomas) vs granuloma faciale vs tinea (KOH+ annular advancing scale — route derm.tinea-dermatophytosis.core.v1) vs drug-induced SCLE (new-drug timeline + anti-Ro + resolution on withdrawal — route derm.drug-eruption.core.v1) vs seborrheic dermatitis (greasy scale + scalp/nasolabial, non-scarring).
Scope: Frame as a CHRONIC/subacute photosensitive autoimmune skin-disease spectrum (ACLE strongly SLE-associated; SCLE anti-Ro + drug-induced subset; DLE scarring/irreversible) managed on a photoprotection-first stepwise ladder. Core tasks: confirm CLE (CLASI activity vs damage), screen EVERY patient for systemic lupus (ACLE/SCLE high-risk), enforce photoprotection + smoking cessation (antimalarial efficacy), and reconcile drugs (drug-induced SCLE). The not-to-miss is active SYSTEMIC lupus hiding behind the rash.

No severity triggers fired against current inputs.

Plan

Regimen axis: **Cutaneous lupus erythematosus — photoprotection-first + stepwise ladder (Lu et al CLE guideline 2021; German S2k 2021)** — step "Step 1 — Universal photoprotection + smoking cessation + vitamin D (every patient, every step, lifelong)".
1. rigorous_broad_spectrum_photoprotection (photoprotection, first line) — German S2k Part 2 (PMID 34338428) — broad-spectrum high-SPF (≥50) UVA+UVB sunscreen + photoprotective clothing + behavioural sun avoidance; UV is the principal CLE trigger and photoprotection prevents lesion induction.
2. smoking_cessation (risk_factor_modification, first line) — Ezra & Jorizzo (PMID 22582908) + Dutz & Werth (PMID 21918570) — smoking blunts antimalarial response in CLE; cessation is central to maximising hydroxychloroquine efficacy and long-term remission.
3. vitamin_d_repletion (supplement, add on) — German S2k Part 2 (PMID 34338428) — rigorous photoprotection predisposes to vitamin-D insufficiency; supplement to maintain adequacy.

Setting playbook (outpatient) — Confirm the CLE subtype (clinico-pathologic, exclude mimics), screen EVERY patient for systemic lupus, enforce photoprotection + smoking cessation, reconcile drugs (drug-induced SCLE), and escalate the photoprotection-first ladder to disease activity/damage and SLE-risk while gating agents on pregnancy/anti-Ro/SLE status (Lu et al CLE guideline PMID 34364171; German S2k Parts 1-2 PMID 34390136 / 34338428)
4. broad-spectrum photoprotection + smoking cessation + vitamin D (foundation) SPF≥50 UVA/UVB topical/behavioural daily, lifelong — All CLE patients, all subtypes/severities (German S2k Part 2 PMID 34338428; Ezra & Jorizzo PMID 22582908) (UV is the principal disease driver; smoking blunts antimalarial response)
5. clobetasol 0.05% / intralesional triamcinolone / tacrolimus 0.1% (face) 0.05% / 2.5–10 mg/mL / 0.1% topical/intralesional pulsed / q4–6wk / BID — Localised low-activity CLE (Lu et al CLE guideline PMID 34364171) (Topical/intralesional corticosteroid + topical calcineurin first-line for localised disease; TCI steroid-sparing on face)
6. hydroxychloroquine ≤5 mg/kg/day ± quinacrine add-on ≤5 mg/kg/day PO once daily — Widespread/severe or topical-refractory CLE; first-line systemic for ALL subtypes (Lu et al CLE guideline PMID 34364171) (Antimalarial first-line systemic; baseline+annual ophthalmology; smoking-cessation maximises response)
7. methotrexate / MMF / thalidomide (refractory DLE) / belimumab / anifrolumab; withdraw culprit if drug-induced SCLE agent-specific PO/SC/IV agent-specific — Antimalarial-refractory CLE or widespread CLE with active SLE (Lu et al CLE guideline PMID 34364171) (Second/third-line immunosuppression; belimumab/anifrolumab for SLE-active widespread CLE; drug-induced SCLE → withdraw culprit)

Non-pharmacologic actions:
- Photoprotection education: broad-spectrum SPF≥50, photoprotective clothing, behavioural sun avoidance, UV-window/fluorescent counsel (German S2k Part 2 PMID 34338428)
- Structured smoking-cessation referral — framed as a treatment lever for antimalarial efficacy (Ezra & Jorizzo PMID 22582908)
- Early aggressive treatment of active DLE/scalp lesions to prevent irreversible scarring/dyspigmentation/scarring alopecia (German S2k Part 2 PMID 34338428)
- Drug-induced-SCLE culprit withdrawal + medication-list documentation (He & Sawalha PMID 29870500)
- Skin-of-colour dyspigmentation + QoL counselling; camouflage/reconstruction only after activity controlled (Creadore et al PMID 32360722)

AVOID / contraindication checks:
- Hydroxychloroquine retinopathy screen and dose cap (Lu et al CLE guideline PMID 34364171 / German S2k Part 2 PMID 34338428 — ≤5 mg/kg actual weight/day; baseline + annual ophthalmologic screening; never co administer with chloroquine)
- Thalidomide lenalidomide mycophenolate methotrexate teratogenic pregnancy contraindicated (Lu et al CLE guideline PMID 34364171 — thalidomide/lenalidomide under strict REMS; hydroxychloroquine is the pregnancy compatible systemic)
- Thalidomide lenalidomide peripheral neuropathy surveillance (dose dependent, partly irreversible — neurologic monitoring; lenalidomide adds thrombotic risk)
- Anti ro ssa pregnancy neonatal lupus congenital heart block (German S2k Part 2 PMID 34338428 — fetal cardiology + maternal fetal medicine; continue hydroxychloroquine)
- Smoking reduces antimalarial efficacy (Ezra & Jorizzo PMID 22582908; Dutz & Werth PMID 21918570 — cessation is a treatment lever, not optional advice)
- Strict uv sun avoidance and photoprotection (German S2k Part 2 PMID 34338428 — UV is the principal disease driver)
- Dapsone check g6pd before start (haemolysis / methaemoglobinaemia risk)
- Drug induced scle withdraw culprit not escalate immunosuppression (He & Sawalha PMID 29870500)

Monitoring

Regimen monitoring:
- CLASI activity plus CLASI damage each visit (German S2k Part 1 PMID 34390136 — activity guides escalation; damage captures irreversible scarring/dyspigmentation/alopecia early)
- hydroxychloroquine: baseline + annual ophthalmologic retinopathy screen; dose ≤5 mg/kg/day (Lu et al CLE guideline PMID 34364171)
- SLE surveillance: CBC, creatinine + urinalysis, complement/anti-dsDNA periodically — esp. ACLE/SCLE (Cochrane PMID 33687069; Zhou et al PMID 32746644)
- methotrexate: CBC + LFT periodic + folic acid co-prescribed (Lu et al CLE guideline PMID 34364171)
- thalidomide/lenalidomide: REMS pregnancy testing + peripheral-neuropathy surveillance (Lu et al CLE guideline PMID 34364171)
- mycophenolate: CBC + LFT periodic; pregnancy avoidance (Lu et al CLE guideline PMID 34364171)
- belimumab/anifrolumab: infection (zoster/respiratory) surveillance; CLASI skin response by 8-24 wk (Morand PMID 38288923; Bruce PMID 36639192; Zen PMID 37109077)
- smoking-cessation adherence + photoprotection adherence (antimalarial efficacy lever) (Ezra & Jorizzo PMID 22582908)

Setting (outpatient) monitoring:
- Reassess CLASI activity + CLASI damage each visit per agent (German S2k Part 1 PMID 34390136)
- HCQ baseline + annual ophthalmology; SLE surveillance CBC/renal/complement esp. ACLE/SCLE; immunosuppressant class labs on schedule (Lu et al CLE guideline PMID 34364171)

Follow-up plan: Chronic-disease maintenance: lifelong rigorous photoprotection + smoking-cessation reinforcement (central to antimalarial response), HCQ annual ophthalmology, longitudinal SLE-progression surveillance (ACLE/SCLE highest — re-screen renal/heme/serology periodically), early treatment of any new DLE activity to prevent irreversible scarring/dyspigmentation/scarring alopecia, skin-of-colour dyspigmentation + QoL support, anti-Ro pregnancy counselling/planning, and step-down/step-up criteria. Dermatology continuity for any systemic agent; rheumatology co-management if systemic lupus.
- Close-out criterion: photoprotection + smoking-cessation + SLE surveillance + early-scarring-prevention + QoL + pregnancy counselling documented

Monitoring phase: Disease: CLASI activity + CLASI damage at each visit to judge response and capture irreversible scarring early (antimalarial effect over 6-12 wk; anifrolumab/belimumab skin response over 8-24 wk). Drug safety: hydroxychloroquine → baseline + annual ophthalmologic retinopathy screen, dose cap ≤5 mg/kg/day; methotrexate → CBC/LFT periodic + folic acid; mycophenolate → CBC/LFT; thalidomide/lenalidomide → REMS pregnancy testing + peripheral-neuropathy surveillance; SLE surveillance → CBC, creatinine/urinalysis, complement/anti-dsDNA (panel.cbc/renal/inflammation), esp. ACLE/SCLE. Track smoking-cessation adherence + photoprotection.

Disposition

Current setting: outpatient — Confirm the CLE subtype (clinico-pathologic, exclude mimics), screen EVERY patient for systemic lupus, enforce photoprotection + smoking cessation, reconcile drugs (drug-induced SCLE), and escalate the photoprotection-first ladder to disease activity/damage and SLE-risk while gating agents on pregnancy/anti-Ro/SLE status (Lu et al CLE guideline PMID 34364171; German S2k Parts 1-2 PMID 34390136 / 34338428)

Disposition criteria:
- Continue ladder + photoprotection + smoking-cessation + derm ± rheum follow-up if responding (Lu et al CLE guideline PMID 34364171)
- Step up the ladder if an adequate antimalarial trial fails after photoprotection/smoking/adherence optimisation
- Route OUT: active SLE → rheum.sle.core.v1; anti-Ro pregnancy → MFM/fetal cardiology; drug-induced SCLE → withdraw culprit + derm.drug-eruption.core.v1

Escalation triggers (move to higher acuity):
- CLE with active SLE organ involvement (nephritis/cytopenia/serositis) → urgent rheumatology + systemic immunosuppression (route rheum.sle.core.v1)
- SCLE in pregnancy with anti-Ro/SSA → fetal cardiology + maternal-fetal medicine (neonatal lupus / CHB)
- HCQ retinopathy signal → ophthalmology + stop hydroxychloroquine
- Rapidly scarring DLE / scalp scarring alopecia → aggressive early systemic therapy (irreversible damage)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] CLE rash accompanied by active SLE organ involvement — lupus nephritis, cytopenia, serositis, or systemic flare (esp. ACLE/SCLE)
- [SEVERE] Active discoid LE with rapidly progressive scarring / dyspigmentation, or expanding scalp cicatricial (scarring) alopecia
- [SEVERE] SCLE (or any CLE) in a pregnant/conceiving patient with anti-Ro/SSA positivity

Citations

- Lu et al, J Autoimmun 2021 (ADA/AADV/CSD evidence- and consensus-based guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus; PMID 34364171, DOI 10.1016/j.jaut.2021.102707) + Worm et al German S2k guideline Part 1 (PMID 34390136, DOI 10.1111/ddg.14492) and Part 2 (PMID 34338428, DOI 10.1111/ddg.14491) + Hannon et al Cochrane review of interventions for cutaneous disease in SLE (PMID 33687069, DOI 10.1002/14651858.CD007478.pub2) [PMID:34364171](https://pubmed.ncbi.nlm.nih.gov/34364171/)
- Cited evidence (PMID 34390136) [PMID:34390136](https://pubmed.ncbi.nlm.nih.gov/34390136/)
- Cited evidence (PMID 34338428) [PMID:34338428](https://pubmed.ncbi.nlm.nih.gov/34338428/)
- Cited evidence (PMID 33687069) [PMID:33687069](https://pubmed.ncbi.nlm.nih.gov/33687069/)
- Cited evidence (PMID 22582908) [PMID:22582908](https://pubmed.ncbi.nlm.nih.gov/22582908/)

Last reconciled with current guidelines: 2026-05-22.
References
  • Lu et al, J Autoimmun 2021 (ADA/AADV/CSD evidence- and consensus-based guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus; PMID 34364171, DOI 10.1016/j.jaut.2021.102707) + Worm et al German S2k guideline Part 1 (PMID 34390136, DOI 10.1111/ddg.14492) and Part 2 (PMID 34338428, DOI 10.1111/ddg.14491) + Hannon et al Cochrane review of interventions for cutaneous disease in SLE (PMID 33687069, DOI 10.1002/14651858.CD007478.pub2) — PMID:34364171
  • Cited evidence (PMID 34390136) — PMID:34390136
  • Cited evidence (PMID 34338428) — PMID:34338428
  • Cited evidence (PMID 33687069) — PMID:33687069
  • Cited evidence (PMID 22582908) — PMID:22582908