Derm Cases · Dermatology / Haemato-oncology
OSCE — erythrodermic patient: recognise Sézary syndrome, stage blood involvement, and start multimodal care
An 8-minute OSCE on recognising the Sézary triad, ordering blood flow cytometry/TCR clonality, assigning B2/IVA1 concepts, differentiating erythrodermic MF, and outlining ECP/systemic therapy plus infection control.
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NEET-PGINICETUSMLEPLABMRCP
Prompt
An 8-minute OSCE on recognising the Sézary triad, ordering blood flow cytometry/TCR clonality, assigning B2/IVA1 concepts, differentiating erythrodermic MF, and outlining ECP/systemic therapy plus infection control.
Brief (to candidate)
A 71-year-old man has whole-body redness, scale and severe itch for 10 months, labelled “eczema.” He has generalised lymphadenopathy. You have 8 minutes to suspect Sézary syndrome, plan decisive investigations, stage conceptually, and outline initial multimodal management.
[1]Candidate instructions
- State the diagnostic triad and why chronic erythroderma is not “just eczema.”
- List blood tests (smear, flow cytometry CD7/CD26, Sézary count, CD4:CD8, TCR clone).
- Explain B2 and typical stage IVA1 mapping when viscera are spared.
- Differentiate erythrodermic MF (B0/B1).
- Outline supportive care + ECP/systemic options (including mogamulizumab concept) and infection control.
Examiner checklist (mark each domain / 10)
| Domain | Key actions expected |
|---|---|
| Recognition | Suspects SS: erythroderma + nodes + circulating Sézary cells[1][2] |
| Blood work-up | Orders flow (CD4+ with CD7/CD26 loss), absolute Sézary count, CD4:CD8, TCR clonality ± skin biopsy correlation |
| Staging language | Uses ISCL/EORTC TNMB; B2 blood; classic SS often IVA1 if T4 N0–2 M0 B2[2] |
| Differential | Separates erythrodermic MF (lower blood burden) and non-CTCL erythroderma |
| Therapy outline | Multimodal: skin care, treat infection, ECP, systemic immunomodulators; mogamulizumab in advanced/pretreated MF/SS context; transplant only for selected fit refractory disease[3][4][5] |
| Safety | Flags sepsis risk in erythroderma; urgent derm–haem-onc pathway |
| Communication | Explains chronicity, need for specialist care, realistic prognosis framing |
Model key actions
- Do not discharge as steroid-responsive eczema without blood staging when SS is plausible.[1]
- Confirm B2 before labelling classic SS.[2]
- Start barrier/infection care in parallel with disease-directed therapy.[4]
Common errors
- Treating only with emollients indefinitely.
- Ignoring blood flow cytometry.
- Equating all erythroderma with SS without B2 criteria.
- Offering allo-HSCT as first step for every elderly patient without fitness assessment.
References6ShowHide
- [1]Miyashiro D, Sanches JA. Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management. Frontiers in Oncology, 2023.PMID 37124514
- [2]Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome. Blood, 2007.PMID 17540844
- [3]Kim YH, Bagot M, Pinter-Brown L, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC). Lancet Oncology, 2018.PMID 30100375
- [4]Latzka J, Assaf C, Bagot M, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023. European Journal of Cancer, 2023.PMID 37890355
- [5]Zic JA. Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sézary Syndrome. Dermatologic Clinics, 2015.PMID 26433848
- [6]Kim YH, Bagot M, Pinter-Brown L, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial Lancet Oncol, 2018.PMID 30100375