12-phase flow (12)

1. 1FRAME
   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.
   advance: CLE-spectrum framing set; SLE-screen + drug-induced-SCLE + scarring escape routes noted
2. 2ENTRY
   Recognise the photodistributed CLE eruption and assign the subtype lens — malar/butterfly ACLE vs annular/papulosquamous SCLE vs fixed scarring discoid DLE (+ variants: panniculitis/profundus, chilblain, tumid) vs the drug-induced-SCLE and CLE-with-systemic-features entries. Capture lesion morphology + subtype up front (drives SLE-risk and scarring urgency).
   inputs: lesion_morphology_and_subtype
   actions: workup.chronic_pruritus
   advance: entry trigger present; CLE subtype lens assigned
3. 3CONTEXT
   Build the diagnosis + treatment context: photodistribution + UV-provocation pattern, the SYSTEMIC-lupus organ screen (arthralgia/serositis/nephritis/cytopenia — at every visit; ACLE/SCLE high-risk), drug reconciliation for the drug-induced-SCLE subset (PPI, thiazide/ACE-i/CCB, terbinafine, anti-TNF, taxane), smoking status (smoking blunts antimalarial efficacy), and a rigorous prior-therapy-adequacy assessment (under-treatment incl. inadequate photoprotection / sub-target HCQ dose is the commonest "failure").
   inputs: photodistribution_and_uv_relation, systemic_lupus_organ_screen, medication_reconciliation_for_drug_induced_scle, smoking_status, prior_topical_systemic_adequacy
   actions: workup.chronic_pruritus
   advance: CLE diagnosis supported; SLE screened; drug-induced subset + smoking + true-adequacy context established
4. 4RED_FLAGS
   CLE with systemic-lupus organ involvement (active nephritis / cytopenia / serositis) → urgent rheumatology + systemic immunosuppression (route rheum.sle.core.v1). SCLE in pregnancy with anti-Ro/SSA → neonatal-lupus / congenital-heart-block risk → fetal cardiology + pregnancy-compatible regimen. Drug-induced SCLE → identify and WITHDRAW the culprit. Rapidly scarring DLE / scalp scarring alopecia → aggressive early therapy (damage is irreversible).
   inputs: anti_ro_ssa_status_and_pregnancy
   actions: panel.cbc, panel.renal, panel.inflammation
   advance: systemic-lupus / anti-Ro-pregnancy / drug-induced / rapidly-scarring red flags screened and escalated/routed if present
5. 5INITIAL_WORKUP
   CLE is a clinico-pathologic diagnosis. Targeted workup: serology (ANA, anti-Ro/SSA, anti-La/SSB, anti-dsDNA, anti-Sm, complement) to stratify subtype + systemic risk; SLE surveillance labs (CBC, creatinine + urinalysis, LFT); pre-systemic readiness (CBC/LFT/renal before MTX/MMF/thalidomide and HCQ baseline ophthalmology). Disease activity vs damage is scored clinically (CLASI activity/damage — schema-blocked as TS calculators, captured narratively in RISK_STRATIFICATION).
   inputs: ana_anti_ro_dsdna_sm_serology, cbc_with_differential, creatinine_and_urinalysis, lft
   actions: panel.cbc, panel.lft, panel.renal
   advance: serology + SLE-surveillance labs sent; pre-systemic safety labs drawn if escalation likely
6. 6BRANCHING_WORKUP
   Photodistributed-eruption decision tree: lesional biopsy + direct immunofluorescence (lupus band — interface dermatitis + granular junctional IgG/C3) when clinical/serologic picture is indeterminate or to separate from a confident mimic; photoprovocation testing where available for SCLE; serologic pivots (anti-Ro SCLE; anti-dsDNA/anti-Sm → SLE). Malar sparing nasolabial folds → ACLE not rosacea (route derm.rosacea.core.v1); papulosquamous + biopsy → SCLE vs psoriasis (route derm.psoriasis.core.v1); oral/scalp interface overlap → lichen planus (route derm.lichen-planus.core.v1); new-drug-linked annular → drug-induced SCLE (route derm.drug-eruption.core.v1); KOH+ annular advancing scale → tinea (route derm.tinea-dermatophytosis.core.v1).
   inputs: lesional_biopsy_with_dif_lupus_band
   actions: workup.chronic_pruritus
   advance: CLE subtype confirmed clinico-pathologically OR an alternative dermatosis assigned + routed; systemic lupus screened
7. 7DIFFERENTIAL
   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).
   inputs: malar_vs_centrofacial_pattern
   advance: single best CLE subtype selected; mimics excluded; drug trigger reconciled; systemic lupus assessed
8. 8RISK_STRATIFICATION
   Stratify on (a) CLE activity vs DAMAGE (CLASI activity/damage scored clinically — schema-blocked as registry calculators, captured narratively): localised low-activity → topical/intralesional; widespread/active or topical-refractory → systemic antimalarial; refractory → Step 4. (b) SLE-progression risk by subtype: ACLE/SCLE high → tighter systemic surveillance + rheum co-management; DLE lower but scalp/disseminated DLE still escalates. (c) Scarring trajectory: rapidly scarring DLE / scalp scarring alopecia is irreversible → treat early and aggressively even at modest BSA.
   inputs: scarring_and_scalp_alopecia_extent
   advance: activity-vs-damage tier + SLE-risk tier + scarring-urgency + escalation decision assigned
9. 9TREATMENT
   PHOTOPROTECTION-FIRST always (broad-spectrum UVA/UVB SPF≥50, behavioural sun avoidance, photoprotective clothing) + smoking cessation (improves antimalarial response) + vitamin D + the stepwise ladder. Step 2 topical: high-potency / intralesional corticosteroid, topical calcineurin inhibitor for face/folds. Step 3 systemic first-line: hydroxychloroquine weight-based ≤5 mg/kg/day (baseline + annual ophthalmology), ± quinacrine add-on or chloroquine. Step 4 refractory: methotrexate, mycophenolate, short systemic-steroid bridge, belimumab (widespread CLE with active SLE), anifrolumab (anti-IFNAR / type-I IFN), thalidomide or lenalidomide (refractory DLE — REMS teratogen + neuropathy), dapsone (selected, esp. bullous LE). Drug-induced SCLE → WITHDRAW the culprit (mainstay). Gating: pregnancy/anti-Ro → continue HCQ, avoid thalidomide/lenalidomide/MMF/MTX, fetal cardiology; SLE organ involvement → systemic immunosuppression with rheumatology.
   inputs: pregnancy_lactation, age
   advance: photoprotection + smoking-cessation + appropriate ladder step started; agent gated on pregnancy/anti-Ro/SLE/age; culprit withdrawn if drug-induced
10. 10DISPOSITION
    Almost entirely outpatient/derm-clinic, co-managed with rheumatology when systemic lupus is present. Route OUT: active SLE organ involvement → rheum.sle.core.v1 (urgent if nephritis/cytopenia/serositis); anti-Ro pregnancy → maternal-fetal medicine + fetal cardiology; drug-induced SCLE → withdraw culprit + route derm.drug-eruption.core.v1; refractory DLE for cosmetically disfiguring damage → reconstructive options after activity controlled. Systemic-therapy initiation/monitoring (antimalarial, immunosuppressant, biologic) via dermatology ± rheumatology.
    advance: disposition documented; SLE / anti-Ro-pregnancy / drug-induced routed; derm ± rheum follow-up arranged
11. 11MONITORING
    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.
    inputs: cbc_with_differential, creatinine_and_urinalysis
    actions: panel.cbc, panel.lft, panel.renal
    advance: CLASI activity/damage reassessed at the agent-appropriate interval; drug-class + SLE-surveillance labs on schedule
12. 12FOLLOWUP
    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.
    inputs: systemic_lupus_organ_screen, smoking_status
    actions: workup.chronic_pruritus
    advance: photoprotection + smoking-cessation + SLE surveillance + early-scarring-prevention + QoL + pregnancy counselling documented