derm.cutaneous-t-cell-lymphoma.core.v1PRODUCTION

derm.cutaneous-t-cell-lymphoma.core.v1

Cutaneous T-cell lymphoma — mycosis fungoides & Sézary syndrome (dermatology lens)

dermatologysubacutechronicadult

Hard-required inputs

0 / 10

Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Frame as the NOT-TO-MISS indolent cutaneous lymphoma that classically masquerades as "treatment-resistant eczema/psoriasis" for years. The dermatology engine owns RECOGNITION → repeat steroid-washed skin biopsy with immunophenotype + TCR clonality → ISCL/EORTC TNMB staging incl. blood (Sézary) → stage-directed therapy (skin-directed first for early disease), and ROUTES advanced/systemic/transplant care by engine_id to hematology-oncology shared-care (heme.cutaneous-lymphoma-systemic.core.v1). This is the engine derm.atopic-dermatitis / derm.psoriasis / derm.vitiligo route to when an "eczema/psoriasis" resists adequate therapy or is atypical. Early IA disease has near-normal life expectancy; advanced/Sézary prognosis is grim — the failure mode is years of immunosuppressing an undiagnosed lymphoma (ISCL/EORTC PMID 17540844; Agar PMID 20855822).

Inputs

Actions

Advance rule

Set

Advance when

derm-scope set; resistant-eczema recognition + systemic/heme-onc route noted by engine_id

Patient inputs (18)

prior_topical_steroid_phototherapy_exposure*history • BRANCHING_WORKUP

not present

Recent topical/systemic corticosteroid or phototherapy blunts the epidermotropic infiltrate and lowers biopsy yield — must be held ≥2 wk before serial biopsy (ISCL/EORTC PMID 17540844)

lesion_distribution_and_morphology*symptom • CONTEXT

Fixed non-sun-exposed "bathing-trunk" patches/plaques, poikiloderma, varied morphology, asymmetric atrophic patches argue MF over symmetric flexural eczema or extensor psoriasis (ISCL/EORTC PMID 17540844)

pruritus_severity_and_qol*symptom • CONTEXT

Pruritus is the dominant symptom and a key patient-reported outcome (often intractable in erythrodermic/Sézary disease); drives supportive care and step-up decisions (EORTC 2023 PMID 37890355)

duration_and_treatment_resistance_history*history • ENTRY

not present

Years of a fixed, waxing-waning "eczema/psoriasis" that never truly clears on adequate topicals/phototherapy is the single strongest recognition signal (median diagnostic delay several years) (ISCL/EORTC PMID 17540844; Agar PMID 20855822)

skin_biopsy_immunophenotype_tcr_clonality*history • INITIAL_WORKUP

not present

Definitive diagnosis — atypical epidermotropic cerebriform T-cells + Pautrier microabscess, aberrant immunophenotype (CD7/CD26 loss, CD4:CD8 skew), and T-cell-receptor clonality; the ISCL early-MF point algorithm integrates clinical + histopathologic + molecular + immunopathologic criteria (ISCL/EORTC PMID 17540844)

cbc_with_differential_and_sezary_flow*lab • INITIAL_WORKUP

Peripheral-blood flow cytometry / Sézary-cell count defines the B (blood) class: B0 none, B1 low, B2 ≥1000/µL Sézary cells or clone with CD4:CD8 ≥10 — B2 erythrodermic disease = Sézary syndrome (ISCL/EORTC PMID 17540844; MAVORIC PMID 30100375)

lymphadenopathy_and_visceral_signs*symptom • RED_FLAGS

Palpable / enlarged nodes and organomegaly define N and M classes and trigger nodal biopsy + stage-directed imaging (ISCL/EORTC PMID 17540844)

large_cell_transformation_signs*symptom • RED_FLAGS

Rapidly growing tumour/nodule, ulceration, or accelerated progression in established MF suggests large-cell transformation (CD30± large cells >25%) — a major adverse prognostic shift requiring re-biopsy and escalation (Agar PMID 20855822)

erythroderma_and_skin_failure_signs*symptom • RED_FLAGS

>80% confluent erythema with thermoregulatory/fluid compromise, exfoliation, and superinfection risk = skin failure — erythrodermic MF/Sézary, admission threshold (EORTC 2023 PMID 37890355)

secondary_infection_sepsis_signs*symptom • RED_FLAGS

Staphylococcus aureus colonisation/bacteraemia is the leading cause of death in advanced MF/SS; honey-crust, pustules, weeping, fever, or sepsis features mandate urgent anti-staphylococcal therapy (EORTC 2023 PMID 37890355)

staging_imaging_by_stageimaging • BRANCHING_WORKUP

not present

CT or PET-CT for nodal/visceral staging is stage-directed — not for limited early patch/plaque disease, indicated for tumour-stage, erythrodermic, lymphadenopathic, or B2 disease (ISCL/EORTC PMID 17540844)

cd30_expression_and_subtypehistory • BRANCHING_WORKUP

not present

CD30 immunostain on biopsy gates brentuximab vedotin candidacy (ALCANZA enrolled CD30+ MF/pcALCL) and distinguishes folliculotropic / large-cell-transformed variants with distinct prognosis (ALCANZA PMID 28600132; Agar PMID 20855822)

immunosuppression_or_transplanthistory • CONTEXT

not present

Immunosuppressed/solid-organ-transplant patients can have an aggressive CTCL course and need expedited staging + heme-onc routing; chronic immunosuppression of misdiagnosed CTCL is harmful (EORTC 2023 PMID 37890355)

lftlab • INITIAL_WORKUP

Baseline + on-treatment hepatotoxicity monitoring for methotrexate, bexarotene, and routed systemic agents (EORTC 2023 PMID 37890355)

lipid_panel_and_tshlab • MONITORING

Oral bexarotene predictably causes hypertriglyceridaemia and central hypothyroidism — baseline + on-treatment lipids/TSH with pre-emptive lipid-lowering and thyroxine (Duvic PMID 11331325)

ldhlab • RISK_STRATIFICATION

Elevated LDH is an independent adverse prognostic factor and tracks tumour burden / progression risk in MF/SS (Agar PMID 20855822)

pregnancy_statushistory • TREATMENT

Bexarotene/retinoids, methotrexate, chemotherapy and most systemics are teratogenic — pregnancy restricts management to skin-directed therapy and defers systemics (EORTC 2023 PMID 37890355)

creatininelab • TREATMENT

CKD-EPI 2021 race-free eGFR for methotrexate / routed-chemotherapy renal dosing context (EORTC 2023 PMID 37890355; Inker NEJM 2021)

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

Severity triggers (7)

7 need judgement

informationallife_threateningerythrodermic_mf_sezary_skin_failure
Erythrodermic MF / Sézary syndrome (>80% confluent erythema) with skin failure — thermoregulatory/fluid compromise, exfoliation, ± secondary sepsis (ISCL/EORTC PMID 17540844; EORTC 2023 PMID 37890355)
Trigger could not be auto-evaluated — needs clinician judgement.
informationallife_threateningsecondary_staphylococcus_aureus_sepsis
Honey-crust/pustules/weeping with fever or sepsis features on MF/SS skin — Staphylococcus aureus superinfection/bacteraemia (EORTC 2023 PMID 37890355)
Trigger could not be auto-evaluated — needs clinician judgement.
informationalseverelarge_cell_transformation_or_rapid_progression
Rapidly growing/ulcerated tumour or accelerated progression in established MF — suspected large-cell transformation (CD30± large cells >25%) (Agar PMID 20855822)
Trigger could not be auto-evaluated — needs clinician judgement.
informationalsevereadvanced_nodal_visceral_or_sezary_disease
Tumour-stage (IIB), nodal/visceral (IVA–B), or blood-B2/Sézary disease on staging (ISCL/EORTC PMID 17540844)
Trigger could not be auto-evaluated — needs clinician judgement.
informationalmoderatetreatment_resistant_eczema_psoriasis_re_biopsy_for_ctcl
Adult "eczema" or "psoriasis" resistant to adequate therapy >6–12 months, or atypical/fixed/poikilodermatous — the canonical CTCL recognition trigger (ISCL/EORTC PMID 17540844)
Trigger could not be auto-evaluated — needs clinician judgement.
informationalmoderaterising_sezary_blood_burden
Rising peripheral Sézary-cell count / blood clone (B1→B2) on serial flow cytometry in erythrodermic or blood-involved disease (ISCL/EORTC PMID 17540844; MAVORIC PMID 30100375)
Trigger could not be auto-evaluated — needs clinician judgement.
informationalmoderateoral_bexarotene_metabolic_toxicity
Initiation of oral bexarotene without anticipatory lipid/thyroid management (predictable hypertriglyceridaemia + central hypothyroidism) (Duvic PMID 11331325)
Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

TREATMENToptionalDrives dose adjustment

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Recommended regimen

Cutaneous T-cell lymphoma (MF/SS) — stage-directed ladder (ISCL/EORTC + EORTC 2023; advanced/systemic routed to hematology-oncology)

axis: ctcl_stage_directed_ladderstep 1 - Step 1 — Early-stage (IA–IIA) skin-directed therapy (dermatology-owned; goal is control + QoL, not cure)

Selected step "Step 1 — Early-stage (IA–IIA) skin-directed therapy (dermatology-owned; goal is control + QoL, not cure)" — Limited patch/plaque disease (T1/T2, N0 M0 B0–B1) — most patients; near-normal life expectancy, avoid overtreatment

triamcinolone acetonide
first line
mid_potency_topical_corticosteroid
0.1% cream/ointment • topical • BID to lesions (max: lowest effective potency/duration)
triggers: limited_patch_plaque_disease
EORTC 2023 (PMID 37890355) — topical corticosteroids are a first-line skin-directed option for early-stage MF; potency matched to lesion thickness/site. NOTE: hold ≥2 wk before any diagnostic/repeat biopsy (steroids blunt the epidermotropic infiltrate).
rxcui 10759
clobetasol propionate
first line
high_potency_topical_corticosteroid
0.05% ointment • topical • BID short course (max: avoid prolonged large-BSA use)
triggers: thick_indurated_plaque
EORTC 2023 (PMID 37890355) — superpotent topical corticosteroid for thick/indurated plaques as skin-directed control; same pre-biopsy washout caveat.
rxcui 21245
mechlorethamine
first line
topical_alkylating_nitrogen_mustard
0.016% gel (chlormethine) • topical • once daily to affected skin (max: per labeling; hold for contact dermatitis)
triggers: patch_plaque_mf_skin_directed, topical_corticosteroid_inadequate
EORTC 2023 (PMID 37890355) — topical chlormethine (nitrogen-mustard) gel is a first-line skin-directed therapy for early-stage MF; main adverse effect is cutaneous (allergic/irritant) contact dermatitis.
rxcui 6674
bexarotene (topical)
second line
topical_rxr_retinoid
1% gel • topical • applied to localised lesions, titrated (max: per tolerance/site irritation)
triggers: localised_refractory_patch_plaque
EORTC 2023 (PMID 37890355) — topical bexarotene gel is a skin-directed option for localised early-stage MF lesions; local irritation common.
rxcui 233272
narrowband_uvb_or_puva_phototherapy
first line
phototherapy
triggers: extensive_patch_disease_nbuvb, thicker_plaque_or_pigmented_skin_puva
EORTC 2023 (PMID 37890355) — phototherapy is a first-line skin-directed therapy: nbUVB for patch/thin-plaque disease, PUVA for thicker plaques / deeper involvement; durable responses in early-stage MF.
localized_radiotherapy
first line
radiotherapy
triggers: unilesional_or_localized_tumour, localised_refractory_plaque
EORTC 2023 (PMID 37890355) — MF is exquisitely radiosensitive; localized (spot) radiotherapy is highly effective for unilesional / localized refractory disease and palliation of individual tumours.

outpatient playbook — drug actions (4)

1. topical corticosteroid / topical chlormethine gel / phototherapy (skin-directed, early-stage)
rxcui 6674
chlormethine 0.016% gel once daily (or TCS / nbUVB-PUVA) • topical • once daily / per phototherapy schedule
trigger: Limited early-stage (IA–IIA) patch/plaque MF after biopsy confirmation (EORTC 2023 PMID 37890355)
Skin-directed therapy controls early-stage disease with near-normal life expectancy — goal is control + QoL, not cure
2. oral bexarotene / interferon-α / low-dose methotrexate / TSEBT (refractory-early, extensive)
rxcui 233272
bexarotene 300 mg/m²/day (with anticipatory fenofibrate + levothyroxine) • PO • once daily
trigger: Refractory-early / extensive skin disease (Duvic PMID 11331325; EORTC 2023 PMID 37890355)
Systemic biologic-response modifiers / TSEBT for extensive or skin-directed-refractory disease; mandatory bexarotene lipid/TSH management
3. brentuximab vedotin (CD30+) / mogamulizumab (Sézary/blood) — ROUTED to heme-onc
rxcui 1147320
brentuximab vedotin 1.8 mg/kg IV q3wk • IV • q3wk ≤16 cycles
trigger: Advanced (≥IIB) / erythrodermic / Sézary / CD30+ / transformed disease (ALCANZA PMID 28600132; MAVORIC PMID 30100375)
Targeted systemic therapy by heme-onc; derm co-manages skin + cutaneous toxicity and tracks mSWAT
4. HDAC inhibitor / chemotherapy / ECP / allogeneic SCT — ROUTED to heme-onc
rxcui 877510
romidepsin 14 mg/m² IV d1,8,15 q28d (or vorinostat / gemcitabine / liposomal doxorubicin / ECP / alloSCT) • IV • per oncology protocol
trigger: Relapsed/refractory advanced disease or Sézary; alloSCT for selected transplant-eligible (Whittaker PMID 20697094; EORTC 2023 PMID 37890355)
Later-line systemic / curative-intent transplant by heme-onc; ECP first-line for Sézary; dermatology resumes skin-directed maintenance after routed therapy

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Adult with a fixed, persistent "eczema" or "psoriasis" that never truly clears despite adequate therapy — the canonical CTCL misdiagnosis, routed in from derm.atopic-dermatitis / derm.psoriasis (ISCL/EORTC PMID 17540844; EORTC 2023 PMID 37890355); Fixed, non-sun-exposed "bathing-trunk"-distributed scaly patches/plaques with poikiloderma (atrophy, telangiectasia, dyspigmentation) — classic patch/plaque-stage mycosis fungoides (ISCL/EORTC PMID 17540844); New cutaneous tumour(s) / ulcerated nodule arising in long-standing patch–plaque disease — tumour-stage (T3) or large-cell transformation (Agar PMID 20855822).

Objective

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

Assessment

**Cutaneous T-cell lymphoma — mycosis fungoides & Sézary syndrome (dermatology lens)** (derm.cutaneous-t-cell-lymphoma.core.v1).
Phenotype framing: Terminal eczematous/erythrodermic differential with named pivots: MF vs atopic dermatitis (route derm.atopic-dermatitis.core.v1 — the canonical misdiagnosis; pivot: fixed non-sun-exposed bathing-trunk distribution + poikiloderma + treatment-resistance + atypical clonal epidermotropic biopsy) vs psoriasis (route derm.psoriasis.core.v1 — sharp salmon plaque/silver scale/nail pits pivot) vs nummular eczema (coin lesions, responds to steroids pivot) vs tinea corporis (route derm.tinea-dermatophytosis.core.v1 — KOH+ annular advancing scale pivot) vs drug eruption (temporal drug link pivot) vs large-plaque parapsoriasis (an MF precursor / early form — low re-biopsy threshold pivot) vs erythroderma causes (Sézary vs drug vs erythrodermic psoriasis vs erythrodermic AD — peripheral-blood clonality + Sézary-cell count/B-class + CD4:CD8 pivot) vs CD30+ lymphoproliferative disorders — lymphomatoid papulosis / primary-cutaneous ALCL (waxing-waning self-healing papulonodules vs MF pivot) vs allergic contact dermatitis (geometric margin + patch-test pivot). When an adult "eczema/psoriasis" resists adequate therapy or is atypical, re-biopsy for MF — recognition delay is the dominant harm.
Scope: Frame as the NOT-TO-MISS indolent cutaneous lymphoma that classically masquerades as "treatment-resistant eczema/psoriasis" for years. The dermatology engine owns RECOGNITION → repeat steroid-washed skin biopsy with immunophenotype + TCR clonality → ISCL/EORTC TNMB staging incl. blood (Sézary) → stage-directed therapy (skin-directed first for early disease), and ROUTES advanced/systemic/transplant care by engine_id to hematology-oncology shared-care (heme.cutaneous-lymphoma-systemic.core.v1). This is the engine derm.atopic-dermatitis / derm.psoriasis / derm.vitiligo route to when an "eczema/psoriasis" resists adequate therapy or is atypical. Early IA disease has near-normal life expectancy; advanced/Sézary prognosis is grim — the failure mode is years of immunosuppressing an undiagnosed lymphoma (ISCL/EORTC PMID 17540844; Agar PMID 20855822).

No severity triggers fired against current inputs.

Plan

Regimen axis: **Cutaneous T-cell lymphoma (MF/SS) — stage-directed ladder (ISCL/EORTC + EORTC 2023; advanced/systemic routed to hematology-oncology)** — step "Step 1 — Early-stage (IA–IIA) skin-directed therapy (dermatology-owned; goal is control + QoL, not cure)".
1. triamcinolone acetonide 0.1% cream/ointment topical BID to lesions (mid_potency_topical_corticosteroid, first line) — EORTC 2023 (PMID 37890355) — topical corticosteroids are a first-line skin-directed option for early-stage MF; potency matched to lesion thickness/site. NOTE: hold ≥2 wk before any diagnostic/repeat biopsy (steroids blunt the epidermotropic infiltrate).
2. clobetasol propionate 0.05% ointment topical BID short course (high_potency_topical_corticosteroid, first line) — EORTC 2023 (PMID 37890355) — superpotent topical corticosteroid for thick/indurated plaques as skin-directed control; same pre-biopsy washout caveat.
3. mechlorethamine 0.016% gel (chlormethine) topical once daily to affected skin (topical_alkylating_nitrogen_mustard, first line) — EORTC 2023 (PMID 37890355) — topical chlormethine (nitrogen-mustard) gel is a first-line skin-directed therapy for early-stage MF; main adverse effect is cutaneous (allergic/irritant) contact dermatitis.
4. bexarotene (topical) 1% gel topical applied to localised lesions, titrated (topical_rxr_retinoid, second line) — EORTC 2023 (PMID 37890355) — topical bexarotene gel is a skin-directed option for localised early-stage MF lesions; local irritation common.
5. narrowband_uvb_or_puva_phototherapy (phototherapy, first line) — EORTC 2023 (PMID 37890355) — phototherapy is a first-line skin-directed therapy: nbUVB for patch/thin-plaque disease, PUVA for thicker plaques / deeper involvement; durable responses in early-stage MF.
6. localized_radiotherapy (radiotherapy, first line) — EORTC 2023 (PMID 37890355) — MF is exquisitely radiosensitive; localized (spot) radiotherapy is highly effective for unilesional / localized refractory disease and palliation of individual tumours.

Setting playbook (outpatient) — Recognise the "treatment-resistant eczema/psoriasis" that is actually CTCL, obtain a steroid-washed serial biopsy with immunophenotype + TCR clonality, assign ISCL/EORTC TNMB stage incl. blood (Sézary) class, deliver stage-directed skin-directed therapy for early disease, and route advanced/erythrodermic/Sézary/transformed/transplant disease to hematology-oncology shared-care while retaining skin-directed control and mSWAT surveillance (ISCL/EORTC PMID 17540844; EORTC 2023 PMID 37890355)
7. topical corticosteroid / topical chlormethine gel / phototherapy (skin-directed, early-stage) chlormethine 0.016% gel once daily (or TCS / nbUVB-PUVA) topical once daily / per phototherapy schedule — Limited early-stage (IA–IIA) patch/plaque MF after biopsy confirmation (EORTC 2023 PMID 37890355) (Skin-directed therapy controls early-stage disease with near-normal life expectancy — goal is control + QoL, not cure)
8. oral bexarotene / interferon-α / low-dose methotrexate / TSEBT (refractory-early, extensive) bexarotene 300 mg/m²/day (with anticipatory fenofibrate + levothyroxine) PO once daily — Refractory-early / extensive skin disease (Duvic PMID 11331325; EORTC 2023 PMID 37890355) (Systemic biologic-response modifiers / TSEBT for extensive or skin-directed-refractory disease; mandatory bexarotene lipid/TSH management)
9. brentuximab vedotin (CD30+) / mogamulizumab (Sézary/blood) — ROUTED to heme-onc brentuximab vedotin 1.8 mg/kg IV q3wk IV q3wk ≤16 cycles — Advanced (≥IIB) / erythrodermic / Sézary / CD30+ / transformed disease (ALCANZA PMID 28600132; MAVORIC PMID 30100375) (Targeted systemic therapy by heme-onc; derm co-manages skin + cutaneous toxicity and tracks mSWAT)
10. HDAC inhibitor / chemotherapy / ECP / allogeneic SCT — ROUTED to heme-onc romidepsin 14 mg/m² IV d1,8,15 q28d (or vorinostat / gemcitabine / liposomal doxorubicin / ECP / alloSCT) IV per oncology protocol — Relapsed/refractory advanced disease or Sézary; alloSCT for selected transplant-eligible (Whittaker PMID 20697094; EORTC 2023 PMID 37890355) (Later-line systemic / curative-intent transplant by heme-onc; ECP first-line for Sézary; dermatology resumes skin-directed maintenance after routed therapy)

Non-pharmacologic actions:
- Repeat steroid-washed skin biopsy over time when initial biopsy is non-diagnostic in resistant adult "eczema/psoriasis" (ISCL/EORTC PMID 17540844)
- Phototherapy (nbUVB / PUVA) and localized radiotherapy / TSEBT as skin-directed therapy by stage (EORTC 2023 PMID 37890355)
- Extracorporeal photopheresis for erythrodermic MF / Sézary syndrome (EORTC 2023 PMID 37890355)
- Intensive emollient/barrier + antipruritic skin care and S. aureus decolonisation/surveillance (leading cause of death in advanced disease) (EORTC 2023 PMID 37890355)
- Multidisciplinary cutaneous-lymphoma / heme-onc referral for advanced/erythrodermic/Sézary/transformed disease; allogeneic-transplant evaluation for selected patients (EORTC 2023 PMID 37890355)
- QoL-weighted education: indolent-but-incurable course, avoid overtreatment of early disease (near-normal life expectancy in IA), lifelong surveillance (Agar PMID 20855822)

AVOID / contraindication checks:
- Hold topical and systemic corticosteroids and phototherapy ≥2wk before biopsy (steroids/phototherapy blunt the epidermotropic infiltrate and lower diagnostic yield — repeat biopsy off steroids in resistant "eczema/psoriasis" — ISCL/EORTC PMID 17540844)
- Bexarotene central hypothyroidism and hypertriglyceridemia (anticipatory levothyroxine + fenofibrate and baseline/serial TSH freeT4 + fasting lipids are MANDATORY before and during oral bexarotene — Duvic JCO 2001 PMID 11331325)
- Mechlorethamine contact dermatitis (allergic/irritant contact dermatitis is the principal adverse effect of topical chlormethine gel — counsel, monitor, hold/desensitise — EORTC 2023 PMID 37890355)
- Retinoid chemotherapy and most systemics teratogenic in pregnancy (bexarotene, methotrexate, gemcitabine, anthracycline, brentuximab and most systemics are teratogenic — pregnancy → skin directed therapy only, defer systemics — EORTC 2023 PMID 37890355)
- Live vaccines avoided on systemic immunosuppression or immunotherapy (EORTC 2023 PMID 37890355)
- Do not chronically immunosuppress undiagnosed treatment resistant eczema psoriasis (re biopsy adult disease resistant to adequate therapy or atypical — immunosuppressing undiagnosed CTCL is harmful and delays diagnosis — ISCL/EORTC PMID 17540844)
- Staphylococcus aureus infection is the leading cause of death in advanced disease (low threshold for anti staphylococcal therapy, decolonisation, and the sepsis pathway in erythrodermic/Sézary disease — EORTC 2023 PMID 37890355)

Monitoring

Regimen monitoring:
- mSWAT skin-score + photographs + symptom (pruritus) tracking at each visit for stage-based response (EORTC 2023 PMID 37890355 — mSWAT schema-blocked, captured narratively)
- blood Sézary-cell count / flow cytometry (B0/B1/B2) tracked for blood-involved disease (ISCL/EORTC PMID 17540844; MAVORIC PMID 30100375)
- LDH trend + re-biopsy on clinical change to detect large-cell transformation (independent adverse prognostic factor — Agar PMID 20855822)
- oral bexarotene: fasting lipids + TSH/free-T4 at baseline, ~2–4 wk, then periodically with anticipatory fenofibrate + levothyroxine (Duvic PMID 11331325)
- methotrexate: CBC + LFT periodic + folic acid; interferon: CBC/LFT + depression/flu-like surveillance (EORTC 2023 PMID 37890355)
- routed systemics: brentuximab peripheral-neuropathy (ALCANZA PMID 28600132), mogamulizumab rash/autoimmune/infusion (MAVORIC PMID 30100375), HDACi QT/cytopenia, anthracycline cumulative cardiotoxicity — co-monitored with heme-onc
- Staphylococcus aureus colonisation/infection surveillance throughout — leading cause of death in advanced MF/SS (EORTC 2023 PMID 37890355)

Setting (outpatient) monitoring:
- mSWAT skin score + photographs + pruritus at each visit; blood Sézary-cell count for B1/B2; LDH trend (EORTC 2023 PMID 37890355; ISCL/EORTC PMID 17540844)
- Bexarotene fasting-lipid + TSH/free-T4 schedule; MTX/interferon CBC-LFT; routed-systemic toxicity co-monitoring (Duvic PMID 11331325)
- Re-biopsy on clinical change to detect large-cell transformation; S. aureus infection vigilance (Agar PMID 20855822; EORTC 2023 PMID 37890355)

Follow-up plan: Lifelong dermatology continuity (incurable but often chronic-indolent in early stages): stage-stratified skin exams, mSWAT + blood-Sézary surveillance, intensive skin-barrier + antipruritic + emollient care and S. aureus decolonisation/surveillance (leading cause of death in advanced), patient education on the indolent-but-monitored course and QoL-weighted goals (avoid overtreatment of early disease — most have a normal life expectancy), bexarotene metabolic-monitoring continuity, and re-biopsy + re-stage + heme-onc re-route on transformation/progression/erythroderma/Sézary conversion. Reconcile any systemic-therapy cutaneous toxicity and resume skin-directed control after routed systemic therapy.
- Close-out criterion: lifelong stage-stratified surveillance + mSWAT/blood tracking + skin/infection care + QoL-weighted education + transformation re-entry path documented

Monitoring phase: Disease: stage-based response by mSWAT skin score + photographs at each visit, blood Sézary-cell tracking (CBC/flow) for B1/B2 disease, LDH trend, and surveillance for large-cell transformation (re-biopsy on any clinical change — a major adverse prognostic shift). Drug safety: oral bexarotene → fasting lipids + TSH/free-T4 at baseline, ~2–4 wk, then periodically (anticipatory fenofibrate + levothyroxine); methotrexate → CBC/LFT periodic + folic acid; interferon → CBC/LFT + depression/flu-like surveillance; routed systemics (brentuximab peripheral neuropathy, mogamulizumab MAVORIC-class skin/infusion/autoimmune events) co-monitored. Infection vigilance throughout — Staphylococcus aureus is the leading cause of death in advanced disease.

Disposition

Current setting: outpatient — Recognise the "treatment-resistant eczema/psoriasis" that is actually CTCL, obtain a steroid-washed serial biopsy with immunophenotype + TCR clonality, assign ISCL/EORTC TNMB stage incl. blood (Sézary) class, deliver stage-directed skin-directed therapy for early disease, and route advanced/erythrodermic/Sézary/transformed/transplant disease to hematology-oncology shared-care while retaining skin-directed control and mSWAT surveillance (ISCL/EORTC PMID 17540844; EORTC 2023 PMID 37890355)

Disposition criteria:
- Early-stage (IA–IIA) → outpatient skin-directed therapy + lifelong surveillance; near-normal life expectancy, avoid overtreatment (Agar PMID 20855822)
- Advanced (≥IIB) / erythrodermic / Sézary / transformed → MDT + heme-onc shared-care; systemic/transplant routed to heme.cutaneous-lymphoma-systemic.core.v1 by engine_id
- Admit only for erythrodermic skin failure, secondary sepsis, or aggressive disease needing inpatient systemic therapy

Escalation triggers (move to higher acuity):
- Erythrodermic MF / Sézary with skin failure (thermoregulatory/fluid compromise) → admit + systemic therapy + infection management (EORTC 2023 PMID 37890355)
- Secondary Staphylococcus aureus sepsis → urgent anti-staphylococcal therapy + sepsis pathway (leading cause of death in advanced disease)
- Large-cell transformation / rapid tumour progression → urgent heme-onc + re-biopsy + restage (Agar PMID 20855822)
- Adult "eczema/psoriasis" resistant to adequate therapy >6–12 mo or atypical → re-biopsy for MF before continued chronic immunosuppression (ISCL/EORTC PMID 17540844)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Erythrodermic MF / Sézary syndrome (>80% confluent erythema) with skin failure — thermoregulatory/fluid compromise, exfoliation, ± secondary sepsis (ISCL/EORTC PMID 17540844; EORTC 2023 PMID 37890355)
- [LIFE_THREATENING] Honey-crust/pustules/weeping with fever or sepsis features on MF/SS skin — Staphylococcus aureus superinfection/bacteraemia (EORTC 2023 PMID 37890355)
- [SEVERE] Rapidly growing/ulcerated tumour or accelerated progression in established MF — suspected large-cell transformation (CD30± large cells >25%) (Agar PMID 20855822)

Citations

- ISCL/EORTC revised staging and classification of mycosis fungoides & Sézary syndrome (Olsen et al, Blood 2007; PMID 17540844, DOI 10.1182/blood-2007-03-055749) + EORTC consensus treatment recommendations Update 2023 (Latzka et al, Eur J Cancer; PMID 37890355, DOI 10.1016/j.ejca.2023.113343 — newest authority, incorporates chlormethine/brentuximab/mogamulizumab/pegylated-IFN; supersedes EORTC 2017 update Trautinger et al PMID 28365528, DOI 10.1016/j.ejca.2017.02.027) + ALCANZA brentuximab vedotin (Prince et al, Lancet 2017; PMID 28600132) + MAVORIC mogamulizumab (Kim et al, Lancet Oncol 2018; PMID 30100375) + bexarotene pivotal (Duvic et al, JCO 2001; PMID 11331325) + romidepsin pivotal (Whittaker et al, JCO 2010; PMID 20697094) + MF/SS prognosis-by-stage validation cohort n=1,502 (Agar et al, JCO 2010; PMID 20855822) [PMID:17540844](https://pubmed.ncbi.nlm.nih.gov/17540844/)
- Cited evidence (PMID 37890355) [PMID:37890355](https://pubmed.ncbi.nlm.nih.gov/37890355/)
- Cited evidence (PMID 28365528) [PMID:28365528](https://pubmed.ncbi.nlm.nih.gov/28365528/)
- Cited evidence (PMID 28600132) [PMID:28600132](https://pubmed.ncbi.nlm.nih.gov/28600132/)
- Cited evidence (PMID 30100375) [PMID:30100375](https://pubmed.ncbi.nlm.nih.gov/30100375/)

Last reconciled with current guidelines: 2026-05-22.

References

ISCL/EORTC revised staging and classification of mycosis fungoides & Sézary syndrome (Olsen et al, Blood 2007; PMID 17540844, DOI 10.1182/blood-2007-03-055749) + EORTC consensus treatment recommendations Update 2023 (Latzka et al, Eur J Cancer; PMID 37890355, DOI 10.1016/j.ejca.2023.113343 — newest authority, incorporates chlormethine/brentuximab/mogamulizumab/pegylated-IFN; supersedes EORTC 2017 update Trautinger et al PMID 28365528, DOI 10.1016/j.ejca.2017.02.027) + ALCANZA brentuximab vedotin (Prince et al, Lancet 2017; PMID 28600132) + MAVORIC mogamulizumab (Kim et al, Lancet Oncol 2018; PMID 30100375) + bexarotene pivotal (Duvic et al, JCO 2001; PMID 11331325) + romidepsin pivotal (Whittaker et al, JCO 2010; PMID 20697094) + MF/SS prognosis-by-stage validation cohort n=1,502 (Agar et al, JCO 2010; PMID 20855822) — PMID:17540844
Cited evidence (PMID 37890355) — PMID:37890355
Cited evidence (PMID 28365528) — PMID:28365528
Cited evidence (PMID 28600132) — PMID:28600132
Cited evidence (PMID 30100375) — PMID:30100375