Cutaneous Lymphomas

Name: Cutaneous Lymphomas: Mini Review for Clinicians, Algorithms & Cases
Creator: Dr. Haritha Chiramana
License: https://creativecommons.org/licenses/by-nc-nd/4.0/

Haematological cancers, part 5 · Mycosis fungoides and variants, Sézary syndrome, lymphomatoid papulosis and cutaneous ALCL, SPTCL and rare T-cell types, follicle centre and marginal zone B-cell lymphomas · Skin-directed treatment · Compartment-based systemic therapy · Key points for clinicians · Last updated 17 October 2026

For health professionals. This section uses clinical terms and is general education for doctors, nurses and students, not advice for an individual patient. Patients and families: please read our patient guides instead, Cancer Myth Busters · Understanding your cancer report · Living through cancer treatment, or speak with your own doctor.

Who this page is for, and where to start

Printable PDFs for this moduleMini reviews, slides, algorithms, quick references and the MCQ booklet are on the Printable Teaching Resources page. No PDF downloads are kept on this page.

Printable Teaching Resources →

For your patientsPlain-language Telugu–English answers for patients and families: the Lymphoma Myth Busters (27 myths), symptoms and warning signs and the Telugu handouts.

Open the lymphoma myths →

SurgeonsScreening and workup (03), local therapy with surgery types (05), algorithms (07), cases (11)

Physicians & oncologistsBiomarkers (04), systemic therapy (06), algorithms (07), survivorship (08), cases (11)

Postgraduate residentsOncology and other specialities: case vignettes (11), landmark trials (09) and 0 cited trials (12) for exams and journal clubs

Medical studentsAt a glance (01), presentation and common traps (02), the figures, and follow-up (08)

Prepared and reviewed by Dr. Haritha Chiramana, MD, DM, Senior Medical Oncologist & Hemato-Oncologist, C.R. Reddy Cancer Center, Nellore, Andhra Pradesh, India. She teaches oncology to postgraduates and medical students; colleges and departments may write to info@drharitha.co.in for teaching sessions. Printable Teaching Resources and how to cite → Phone 0861 232 5344 · WhatsApp +91 79896 42272.

01 At a glance

Primary cutaneous lymphomas start in the skin with no disease elsewhere after staging. They behave differently from nodal lymphomas with skin spread, and most are indolent: treating them like systemic lymphoma causes harm.
About 75% are T-cell, 25% B-cell. Mycosis fungoides (MF, about 40% of all) and the CD30+ disorders (lymphomatoid papulosis and primary cutaneous ALCL, about 25%) make up about 90% of cutaneous T-cell lymphomas; follicle centre and marginal zone lymphomas are most B-cell cases.
Diagnosis is clinicopathologic: early MF looks like eczema or psoriasis for years; biopsies (≥4 mm, off topicals, often repeated) are scored with the ISCL/EORTC 4-point system; a T-cell clone alone proves nothing.
Stage MF and Sézary syndrome by TNMB (skin, nodes, viscera, blood by flow cytometry); other cutaneous lymphomas by their own TNM and a PET-CT to exclude systemic disease.
Treatment follows stage and compartment: early MF → skin-directed (topical steroid, mechlorethamine, NBUVB/PUVA, local RT, TSEBT); tumours and LCT → brentuximab vedotin; erythroderma and blood → ECP or mogamulizumab; allogeneic HCT is the only cure for advanced MF/SS.
Indolent B-cell and CD30+ disorders: low-dose RT, excision, topical or intralesional steroid; lymphomatoid papulosis is often just observed; multiagent chemotherapy is rarely right.
India: hypopigmented MF (children, young adults, darker skin) is mistaken for leprosy, vitiligo or pityriasis alba; in one South Indian series ATLL was 17% of cutaneous T-cell lymphomas, so HTLV-1 testing has a place here too.

Figure 1. Cutaneous lymphomas at a glance (UpToDate classification review; Willemze 2019, WHO-EORTC 2018 update).

02 Clinical presentation, common traps & causes

Figure 2. Frequency and 5-year DSS by type (WHO-EORTC 2018 update, Willemze 2019, as tabulated in the UpToDate classification review; <1 shown as <1).

Who gets it, and why

Mycosis fungoides: about 6 per million per year in Europe and the USA (about 4% of NHL); peak age 55–60, men twice as often as women, more common in Black populations; also seen under 35 with the same course. Not inherited.
How MF shows: itchy patches in the "bathing trunk" area (limited in about 30%, generalised patch/plaque in 35%), tumours in about 20%, erythroderma in 15%; also poikiloderma, alopecia, follicular papules, keratosis pilaris-like lesions. Nodes are involved in about 30%; viscera are rare without tumours or erythroderma.
Variants: folliculotropic MF (the commonest variant in adults; early-stage form behaves like early MF, tumour-stage form like tumour MF); pagetoid reticulosis (solitary acral plaque, benign); granulomatous slack skin (rare; second lymphoma risk, often Hodgkin); unilesional MF (curable locally). Hypopigmented MF is the commonest variant in children and darker skin (33% of MF in a Southeast Asian cohort); often CD8+; responds well.
Sézary syndrome: older adults; rapid erythroderma with nodes, severe itch, alopecia and palm/sole keratoderma; median survival 3–4 years.
Lymphomatoid papulosis: 1.2–1.9 per million per year; peak in the fifth decade but any age; crops of papules that necrose and heal in 3–8 weeks; lifelong risk of a second lymphoma.
SPTCL: median age 36 (20% under 20), women twice as often; up to 20% have autoimmune disease (lupus, juvenile arthritis, Sjögren, type 1 diabetes); painless fat nodules on legs and arms.
Cutaneous B-cell lymphomas: PCFCL (median age 51, slight male excess) on scalp, head and trunk; PCMZL in adult men on trunk and arms, linked with tattoo pigment, tick bites and (in Europe) Borrelia.
India: in a 6-year South Indian series, MF was 57.1% of cutaneous T-cell lymphomas, ATLL 17.1%, PTCL-NOS 17.1% and ALCL 2.9%, with poor outcomes in ATLL (Khader 2016^[1]). At KEM Mumbai, hypopigmented MF was found in 0.17 per 100 skin biopsies, mostly in young men, about 3.8 years after onset, and mimicked Hansen's disease (KEM series^[2]).

Common traps

Years of "eczema", "psoriasis" or "fungal infection" on the buttocks and trunk. Persistent patches in sun-protected skin that change size and shape: biopsy, after stopping topicals.
Pale patches treated as leprosy, vitiligo, tinea versicolor or pityriasis alba. Hypopigmented MF needs a biopsy, sometimes several.
Erythroderma called "drug rash" or atopic dermatitis without blood flow cytometry. Sézary syndrome needs flow cytometry and a skin-matched clone.
A clonal T-cell result read as lymphoma. Clones occur in benign dermatoses, drug eruptions and older people; pseudolymphomas (actinic reticuloid, lymphomatoid drug or contact reactions) can look identical under the microscope.
LyP called a high-grade lymphoma and given CHOP. Large CD30+ atypical cells in a self-healing papule are LyP; multiagent chemotherapy is inappropriate.
Fat nodules called panniculitis or lupus profundus for months. SPTCL needs a deep biopsy that includes fat; lupus profundus is painful and has no clonal TCR.
ALK-positive CD30+ skin nodules are usually systemic ALCL in the skin: stage fully.

03 Diagnosis, workup & staging essentials

Making the diagnosis

Biopsy technique: stop all topicals except emollients for 2–4 weeks; punch of at least 4 mm from the most indurated lesion; several biopsies, sometimes over time. Excisional or deep incisional biopsies (with fat) for nodules, SPTCL and B-cell lymphomas; shave biopsies are inadequate. LyP: excise two or more fresh, non-necrotic lesions.
MF: small to medium cerebriform cells along the basal layer and in the epidermis (epidermotropism) without spongiosis; Pautrier microabscesses are specific but present in about 38%; haloed lymphocytes are the most robust clue. Typical phenotype CD2+, CD3+, CD4+, CD5+, CD7−, CD8−; hypopigmented MF is often CD8+. Large cell transformation (LCT): large cells >25% of the infiltrate or forming nodules.
ISCL/EORTC score for early MF (Figure): clinical up to 2, histology up to 2, clonal TCR 1, immunopathology 1; 4 points makes the diagnosis (Pimpinelli 2005^[3]).
Sézary syndrome needs all three: diagnostic (or compatible) skin biopsy; erythroderma over 80% of body surface; B2 blood (≥1000/µL CD4+CD7− or CD4+CD26− cells) with a TCR clone matching the skin (Olsen 2022^[4]).
CD30+ disorders: LyP shows a wedge-shaped mixed infiltrate with large CD30+ cells (type A in about 75%); primary cutaneous ALCL has sheets of large cells with CD30 in more than 75%; both are usually ALK-negative (nuclear ALK → systemic ALCL).
SPTCL: atypical cells rimming individual fat cells; CD3+, CD8+, βF1+, cytotoxic granules; CD4−, CD56−, EBER−. γδ T-cell lymphoma: TCRγδ+, CD56+, usually CD4−CD8−, reaches epidermis and ulcerates.
B-cell: PCFCL is BCL6+, BCL2− (mostly), MUM1−, not graded; PCMZL is BCL2+, BCL6−, CD10− with monotypic cytoplasmic Ig in plasma cells. Strong BCL2, MUM1, FOXP1 and IgM point to leg-type DLBCL.

Figure 3. Diagnosing early MF (Pimpinelli 2005; UpToDate MF diagnosis review).

Figure 4. Cutaneous lymphoma key (UpToDate MF, SPTCL, LyP, rare subtype, PCFCL and PCMZL reviews; simplified).

Before treatment

Area	What to do	Why
Skin	Whole-skin examination; % body surface by lesion type (the patient's palm ≈ 0.5%); photographs	T stage; response baseline
Nodes and organs	Examination and imaging; excise clinically abnormal nodes (FNA is not enough)	N1–N3; Nx if not biopsied
Blood	CBC, chemistry, LDH; flow cytometry (CD4/CD7/CD26, TRBC1) and TCR clone in MF/SS	B0–B2; CLIPI
Viruses	HTLV-1 where relevant; HBV and HCV before methotrexate	ATLL; safe treatment
Imaging	MF: CT or PET-CT for T2b, T3, T4, nodes >1.5 cm, LCT or B2 (else chest X-ray). Other cutaneous lymphomas: PET-CT	M and N stage; primary vs secondary skin disease
Marrow	Only for unexplained cytopenias or equivocal imaging; core biopsy	M1a

Figure 5. MF/SS staging (Olsen 2007 and 2022; Kim 2007 for non-MF cutaneous lymphomas; UpToDate staging review).

04 Biology and biomarkers that change treatment

Marker or feature	What it tells you	What it changes
CD30 on tumour cells	C-ALCL, LyP, many tumour-stage and transformed MF (40–50% of LCT)	Brentuximab vedotin; even low expression may respond (ALCANZA^[5])
Blood class (B0/B1/B2) by flow cytometry + clone	B1 in early MF and B2 in SS carry worse prognosis	Systemic treatment needed; ECP or mogamulizumab for blood
Large cell transformation (>25% large cells)	1.4% in IA–IIA to 67% in IVB; worse survival	Treat as advanced (BV); consider allo-HCT
Folliculotropism	Infiltrate around and in hair follicles; deeper than NBUVB reaches	PUVA or RT for early FMF; tumour-stage FMF as advanced MF
TCRγδ, CD8 with epidermotropism and necrosis	γδ TCL; CD8+ AECTCL	Aggressive: systemic treatment, transplant referral
HAVCR2 (TIM-3) germline variants; HLH	SPTCL with HLH (17%): 5-year survival 46% vs 91%	Urgent HLH work-up and treatment
BCL2, MUM1, FOXP1, IgM, MYD88	Leg-type DLBCL rather than PCFCL	Treat as leg-type DLBCL (aggressive B-cell module)
Class-switched (IgG) vs IgM PCMZL	IgM cases resemble MALT lymphoma and spread outside skin more often	Stage IgM cases carefully

Figure 6. Prognosis (Scarisbrick 2025, CLIPI; Benton 2013, early CLIPi; UpToDate staging review). Values rounded.

Prognostic scores

CLIPI for advanced MF/SS: N3, age over 60, raised LDH and LCT, 1 point each; 5-year OS 63%, 45% and 18% for 0–1, 2 and 3–4 points (Scarisbrick 2025^[6]).
Early-stage CLIPi: male, over 60, plaques, folliculotropic, N1/Nx; 10-year OS 90%, 76% and 49% (Benton 2013^[7]).

05 Local therapy: skin-directed treatment, radiotherapy & surgery

Skin-directed treatment for mycosis fungoides

Topical corticosteroids (high or super-high potency): first choice for stage IA; response in 94% with 63% complete in T1 disease (Zackheim 1998^[8]); expect a response within 3 months; watch atrophy and striae.
Topical mechlorethamine: response in 70–80% of T1 and benefit in 50–70% of T2; the gel was non-inferior to the ointment (CAILS response 59% vs 48%; Lessin 2013^[9]). Topical bexarotene, imiquimod: for localised, refractory lesions only (topical retinoids are too irritating for large areas).
Phototherapy: NBUVB or PUVA for IB–IIA patches and thin plaques; any response 88% vs 91%, complete response 62% vs 74% (Phan 2019^[10]). Taper slowly after clearing; most relapse when maintenance stops but respond again. PUVA reaches folliculotropic disease better; avoid it after multiple skin cancers.

Figure 7. Skin-directed therapy (Zackheim 1998; Lessin 2013; Phan 2019; Thomas 2013; Hoppe 2015; Kaye 1989; UpToDate early-stage MF review). Mechlorethamine bar = midpoint of 70–80%.

Radiotherapy

Local RT for MF plaques and tumours: 8–12 Gy in one to six fractions; a single fraction (mostly 8 Gy) cleared 94% of 270 lesions (Thomas 2013^[11]); 4 Gy gives less durable control. Unilesional MF (curative): 24–30 Gy. Symptomatic nodes: 12–24 Gy, fractionated.
TSEBT: low dose 10–12 Gy over 2–3 weeks (response 88%, complete 27% in pooled phase 2 trials; repeatable; Hoppe 2015^[12], Kamstrup 2015^[13]) or conventional 30–36 Gy over 9–10 weeks. For thick, widespread, symptomatic plaques, tumours and erythroderma; follow with topical, light or a retinoid for at least 6 months. Erythema, desquamation and hair loss are common; needs an experienced centre.
Indolent cutaneous B-cell lymphoma: RT for lesions within one field. PCFCL: response-adapted very low dose (2 × 2 Gy, margin 1–1.5 cm), escalating to 24 Gy if needed; in 535 patients (ILROG) complete response was 91% at any dose, but 5-year local relapse-free survival was 96% with >4 Gy vs 73% with ≤4 Gy (Oertel 2026^[14]). PCMZL: 24 Gy typical, with a margin (ILROG 2015^[15]).

Surgery

Diagnosis: excisional biopsies for nodules, fat and B-cell lesions.
Treatment: excision for solitary PCFCL or PCMZL in sites unsuitable for RT, unilesional MF, acral CD8+ and CD4+ small/medium T-LPD. Skin relapse after excision is common (about 40% in PCFCL and PCMZL series).

06 Systemic therapy: indications & options

Mycosis fungoides, early stage (IA–IIA)

Skin-directed treatment, not systemic: early aggressive chemotherapy with electron beam gave no survival benefit over sequential topical treatment (Kaye 1989^[16]).
IA: topical steroid → mechlorethamine, bexarotene gel, imiquimod or local RT; most patients have several skin-directed courses over a lifetime. IB–IIA thin plaques: steroid, mechlorethamine, NBUVB or PUVA. Thick, widespread, symptomatic plaques: TSEBT.
Add a systemic agent (oral bexarotene or low-dose methotrexate first; interferon, vorinostat, mogamulizumab or BV less often) for extensive or plaque-predominant disease, inadequate control, no access to phototherapy or TSEBT, folliculotropic MF, LCT or B1 blood.
Folliculotropic MF: early form → PUVA, superpotent steroid or local RT (NBUVB alone usually inadequate); tumour form → as stage IIB (Hodak 2016^[17], van Santen 2017^[18]). B1 blood: treat as stage III with B1.
Itch and dry skin: emollients straight after bathing, topical steroids, antihistamines; gabapentin, mirtazapine or aprepitant for refractory itch.

Mycosis fungoides, advanced stage (IIB–IV)

Figure 8. Advanced MF (Prince 2017 and Horwitz 2021, ALCANZA; Kim 2018, MAVORIC; de Masson 2023, CUTALLO; UpToDate advanced MF review).

IIB, few tumours (patches and plaques under 10% BSA): local RT plus topical treatment; a complementary systemic agent (methotrexate, bexarotene, interferon) may help.
IIB, generalised tumours (≥10% BSA), and LCT: brentuximab vedotin. In ALCANZA (CD30+ MF and C-ALCL), BV vs methotrexate or bexarotene: MF response lasting ≥4 months 50% vs 10%, tumour-stage 63% vs 5%, median PFS 16.1 vs 3.5 months; 9% grade ≥3 neuropathy; no survival difference (ALCANZA^[5], final data^[19]). Without BV: romidepsin, pralatrexate, TSEBT, gemcitabine or liposomal doxorubicin.
Stage III (erythroderma, B0–B1) and stage IVA1: manage as Sézary syndrome (below). IVA2 MF: by skin type (erythroderma → as SS; tumours → as IIB/IVB) with RT to symptomatic nodes.
IVB (viscera): BV, romidepsin or pralatrexate (romidepsin 34% overall, 57% blood response in one CTCL series), with RT 8–12 Gy to skin or 12–24 Gy to nodes as needed; gemcitabine (71%; Zinzani 2000^[20]) or liposomal doxorubicin (41%; EORTC 21012^[21]) also work. Avoid relying on BV for B2 blood.
Allogeneic HCT, the only cure: fit patients up to about 65 with LCT, progressive or refractory disease, done at best response. CUTALLO: median PFS 9 vs 3 months vs no transplant (CUTALLO^[22]); CIBMTR 5-year OS 32% (Lechowicz 2014^[23]); EBMT 7-year OS 44%, with most survivors in CR (Duarte 2014^[24]). No autologous HCT (short responses). BV is a safe bridge; mogamulizumab with caution before transplant.

Sézary syndrome

Figure 9. Sézary syndrome (Olsen 2022; Kim 2018, MAVORIC; UpToDate SS diagnosis and treatment reviews).

Stage IVA (no viscera), and marrow-only IVB: extracorporeal photopheresis (with interferon) or mogamulizumab, alone, together or with bexarotene or interferon. ECP is slow and needs a specialised centre; mogamulizumab is avoided in autoimmune disease and is weaker for bulky nodes, tumours and LCT. IVA2: bexarotene plus interferon is also acceptable.
MAVORIC (previously treated MF and SS): mogamulizumab vs vorinostat, median PFS 8 vs 3 months; responses in blood 68% vs 19%, skin 42% vs 16%, nodes 17% vs 4%; SS response 37% vs 2% (MAVORIC^[25]).
IVB with other viscera, LCT, rapid progression or bulky nodes: single-agent gemcitabine or liposomal doxorubicin rather than combination chemotherapy; then the other agent; allogeneic HCT for younger fit patients. Relapse: sequential single agents, low-dose alemtuzumab, pembrolizumab, trials.
Supportive care: itch (emollients, steroids, doxepin at night, gabapentin or pregabalin, mirtazapine, aprepitant or naltrexone); culture crusted erosions; dilute bleach or chlorhexidine baths with topical antibiotic; systemic antibiotics (think MRSA) for infection; watch for disseminated herpes in erythroderma.

CD30+ lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous ALCL

Figure 10. CD30+ disorders (Kempf 2011 consensus; Melchers 2020; WHO-EORTC 2018; UpToDate LyP review).

LyP: treatment relieves symptoms but does not change the course or the risk of a second lymphoma. Few lesions → observe or a super-potent topical steroid. Many, symptomatic or on face and hands → low-dose weekly methotrexate (with folic acid; often needs maintenance; liver and blood monitoring), then PUVA (or NBUVB/UVA1) if methotrexate fails or is contraindicated. Multiagent chemotherapy is inappropriate (Kempf 2011^[26]).
Children with LyP: often no treatment; topical steroids or NBUVB when needed; low-dose methotrexate second.
Biopsy any lesion >2 cm that has not regressed in 8 weeks to look for primary cutaneous ALCL; nuclear ALK or nodes → systemic ALCL (T-cell module).
Lifelong follow-up: 15.5% of 504 LyP patients developed another haematological cancer (mostly MF or ALCL) over a median of 10 years; deaths from it are uncommon (Melchers 2020^[27], Bekkenk 2000^[28]).

Primary cutaneous anaplastic large cell lymphoma

Figure 11. Primary cutaneous ALCL (Million 2016, ILROG; Prince 2017 and Horwitz 2021, ALCANZA; Bekkenk 2000; Hapgood 2017; Melchers 2018; UpToDate PC-ALCL review).

Who and how: about 8–10% of cutaneous lymphomas; median age 61, slightly more men (SEER). Solitary or grouped nodules, usually on the upper body, growing over weeks to months and often ulcerating; up to half regress partly, rarely completely. About a quarter had two or more sites in the Dutch registry.
Pathology: dense dermal sheets of large anaplastic cells with CD30 on ≥75%; most CD4+ with variable loss of CD2, CD3, CD5; ALK negative and usually EMA negative (both often positive in systemic ALCL); DUSP22::IRF4 rearrangement in up to half, with an indolent course. Exclude ATLL (CD30+ anaplastic, ALK−: test HTLV-1), CD30+ transformed MF, Hodgkin (CD15+) and reactive CD30+ infiltrates.
Staging: examination and blood tests; CT or PET-CT if extracutaneous disease is suspected; biopsy enlarged draining nodes (inflammation is common); marrow only for multifocal tumours, cytopenias or proven extracutaneous disease. Involvement of draining nodes alone does not worsen prognosis.
Localised, no node involvement: excision or involved-site RT, not both, and no chemotherapy; low-dose RT (≤20 Gy) appears as effective as higher doses. ILROG series (56 patients): local control 98%, complete response 95%, responses from 6 Gy (Million 2016^[29], Smith 2017^[30]). With involved draining nodes: ISRT to skin and nodes.
Multifocal: asymptomatic, no nodes → observe (10–15% regress; wait 4–6 weeks for a large lesion, then excise or irradiate if it persists); nodes or systemic symptoms → brentuximab vedotin. In ALCANZA (31 C-ALCL patients) BV beat methotrexate or bexarotene: response ≥4 months 75% vs 20%, CR 31% vs 7%, median PFS 28 vs 5 months; neuropathy improved or resolved in 86% with longer follow-up (ALCANZA^[5], final data^[19]). Oral methotrexate is an alternative; CHOP-type chemotherapy gives brief responses (Melchers 2018^[31]).
Widespread nodal or visceral disease (beyond nodes draining the skin): treat as systemic ALCL (T-cell module).
Relapse: a localised skin relapse → observe first (some regress); if it persists beyond about 2 months or causes symptoms, RT, excision, or super-potent topical or intralesional steroid; multiple skin relapses → BV again if not given in the last 6 months, no progression on it and no neuropathy (88% response on retreatment in systemic ALCL), or methotrexate or bexarotene; widespread or after BV → sequential single agents.
Outlook: disease-related 5-year survival 95% (Dutch registry; Bekkenk 2000^[28]), yet skin relapse in about 40% within 5 years and systemic lymphoma in 14% (Hapgood 2017^[32]); SEER 5- and 10-year OS 81% and 62% (SEER^[33]). Worse with leg lesions (5-year DSS 76% vs 96%; Miyagaki 2023^[34]), five or more lesions (extracutaneous relapse 56% vs 20%), age over 60 and chemotherapy use.

SPTCL and the rare cutaneous T-cell lymphomas

Figure 12. Rare subtypes (Willemze 2008; Gayden 2018; Toro 2003; Berti 1999; Kempf 2022; UpToDate SPTCL and rare-subtype reviews).

SPTCL: mostly indolent (5-year OS about 80%); spread outside the fat is rare and some cases regress. Prognosis is driven by HLH (5-year survival 46% vs 91%), linked to germline HAVCR2 (TIM-3) variants (Willemze 2008^[35], Gayden 2018^[36]). IPI and PIT put most patients in low risk and miss the HLH effect.
Primary cutaneous γδ T-cell lymphoma: ulcerated plaques and tumours on the limbs, often with fat involvement (worse); resistant to multiagent chemotherapy; median survival about 15 months (Toro 2003^[37]).
CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma: eruptive necrotic papules and tumours; chemotherapy responses disappointing; median survival under 2 years; durable remissions after allogeneic HCT in selected patients (Berti 1999^[38]). Distinguish from CD8+ MF, which behaves like ordinary MF.
SPTCL mimics: lupus panniculitis (lupus profundus) favours the face, scalp, upper arms, breasts, upper trunk, buttocks and thighs, is usually painful, heals with marked atrophy, may have discoid lupus on top, shows lymphoid follicles and has no clonal TCR; "cytophagic histiocytic panniculitis" (macrophages eating blood cells, "beanbag cells") can occur with SPTCL or without any lymphoma.
Acral CD8+ T-LPD (usually the ear) and CD4+ small/medium T-LPD (solitary face, neck or upper trunk nodule; 8–12% of CTCL): excision, RT or intralesional steroid; some regress after biopsy (Kempf 2022^[39]).

SPTCL treatment: not yet covered in this module

Treatment of SPTCL will be added after review of a dedicated source. Plan treatment with the lymphoma team; manage suspected HLH urgently (section 06 and Table D).

Primary cutaneous follicle centre and marginal zone lymphomas

Figure 13. Cutaneous B-cell lymphomas (Senff 2008; Oertel 2026, ILROG; UpToDate PCFCL and PCMZL reviews).

PCFCL (EORTC/ISCL, ILROG, NCCN; Senff 2008^[40]): localised and symptomatic or cosmetically troublesome → RT; not troublesome → topical or intralesional steroid or observation. Lesions beyond one field → RT to up to three symptomatic sites, steroids for the rest. Extensive symptomatic disease → single-agent rituximab; combination chemotherapy only for very extensive disease or rituximab failure. About 10% spread outside the skin over 10 years.
PCMZL: lesions in one field → RT (24 Gy) or excision; asymptomatic multifocal → observe; symptomatic multifocal → intralesional triamcinolone, low-dose RT or excision rather than chemotherapy. Five-year survival 98–100%. Borrelia testing where relevant (Europe).
Relapse (both): about a third relapse after RT, mostly in skin. Outside the field → RT; inside the field → excision or rituximab. Extracutaneous PCFCL → treat as follicular lymphoma (indolent B-cell module).

India now (October 2026)

Indian data are web-verified: a South Indian series (MF 57%, ATLL 17% of cutaneous T-cell lymphomas; Khader 2016) and the KEM Mumbai hypopigmented MF series (mimicking Hansen's disease).
Availability and cost in India of mechlorethamine gel, topical and oral bexarotene, PUVA/NBUVB units, TSEBT, extracorporeal photopheresis, mogamulizumab, brentuximab vedotin, romidepsin and pralatrexate, and access to allogeneic transplant, to be confirmed by the treating team.

Quick reference (also as a printable PDF on the Printable Teaching Resources page)

Table A. Diagnosis and staging tests and what they change

Test	When	What it changes	Pitfalls
Skin punch biopsy ≥4 mm (most indurated lesion); often several over time	Every suspected MF	ISCL/EORTC score; variant (folliculotropic, LCT)	Topicals in the last 2–4 weeks blur the picture
Excisional or deep incisional biopsy including fat	Nodules, SPTCL, B-cell lymphomas, LyP (2 fresh lesions)	Entity	Shave biopsies are inadequate
IHC: CD2, CD3, CD4, CD5, CD7, CD8, CD30, ALK, TCRβF1/γδ, CD56, cytotoxic markers; B-cell panel with BCL2, BCL6, MUM1, CD10, light chains	Every biopsy	Names the entity; CD30 → BV option	Read with the clinical picture: pseudolymphomas mimic all of these
TCR (or IgH) clonality, skin and blood	Equivocal histology; erythroderma; staging B and N	1 ISCL point; B2 needs a skin-matched clone	Clones occur in benign disease and with age
Blood flow cytometry (CD4+CD7−, CD4+CD26−; TRBC1)	Every MF/SS staging	B0/B1/B2; Sézary syndrome	Lymphopenia: use percentages
CBC, chemistry, LDH; HTLV-1 serology if from an endemic area or flower cells/high calcium	Baseline	CLIPI; ATLL excluded	ATLL was 17% of cutaneous T-cell lymphomas in one South Indian series
CT or PET-CT	MF with T2b, T3, T4, nodes >1.5 cm, LCT or B2; all non-MF cutaneous lymphomas	N and M stage; primary vs secondary skin lymphoma	Chest X-ray if no CT/PET in limited MF
Node excision; marrow biopsy only if cytopenias or equivocal PET	Enlarged node; suspected systemic disease	N3 (IVA2); M1	FNA cannot grade nodal involvement

Table B. Treatment by setting

Setting	Standard choice	Key evidence	Notes
MF IA	Potent topical steroid; then mechlorethamine, bexarotene gel, imiquimod, local RT	Zackheim 1998^[8]	Rotate skin-directed options before systemic drugs
MF IB–IIA, thin plaques	Steroid, mechlorethamine, NBUVB or PUVA	Phan 2019^[10]	PUVA: more CR; both need maintenance tapering
MF IB–IIA, thick widespread plaques	TSEBT (low dose 10–12 Gy), then adjuvant topical, light or retinoid	Hoppe 2015^[12]	Add systemic agent if B1, folliculotropic or LCT
MF IIB, few tumours	Local RT 8–12 Gy + topicals	Thomas 2013^[11]	Electrons or low-energy x-rays
MF IIB, generalised tumours; LCT	Brentuximab vedotin	ALCANZA^[5]	Neuropathy; TSEBT after response
MF III and Sézary IVA	ECP ± interferon, or mogamulizumab	MAVORIC^[25]	Treat itch and Staphylococcus too
MF IVB; SS with M1b, LCT or bulky nodes	BV, romidepsin or pralatrexate (MF); gemcitabine or liposomal doxorubicin (SS)	Zinzani 2000^[20], EORTC 21012^[21]	RT 12–24 Gy to symptomatic nodes
Advanced MF/SS, fit, ≤65	Allogeneic HCT at best response	CUTALLO^[22]	No autologous HCT
LyP	Observe or topical steroid; low-dose methotrexate; PUVA	Kempf 2011^[26]	No multiagent chemotherapy
PCFCL or PCMZL, localised	RT (PCFCL 4 Gy response-adapted to 24 Gy; PCMZL 24 Gy) or excision	ILROG 2026^[14]	Leg site PCFCL: worse outlook
PCFCL extensive; PCMZL multifocal	PCFCL: rituximab; PCMZL: observe, intralesional steroid, low-dose RT	Senff 2008^[40]	Chemotherapy rarely
Acral CD8+ or CD4+ small/medium T-LPD	Excision, RT or intralesional steroid	Kempf 2022^[39]	Behave like pseudolymphoma
Primary cutaneous ALCL, localised	Excision or involved-site RT (≤20 Gy appears enough)	Million 2016^[29]	No added RT after excision; no chemotherapy
C-ALCL, multifocal with nodes or symptoms	Brentuximab vedotin (methotrexate if neuropathy)	ALCANZA^[5]	Asymptomatic multifocal: observe first
SPTCL	Not yet covered in this module	—	Plan with the lymphoma team; HLH is urgent

Table C. Systemic options: evidence and best fit

Option	Best for	Evidence	Watch for
Brentuximab vedotin	CD30+ MF (tumours, LCT, nodes); multifocal C-ALCL	ALCANZA^[5]: MF response ≥4 months 50% vs 10%; C-ALCL 75% vs 20%, PFS 28 vs 5 months	Peripheral neuropathy; not relied on for B2 blood
Mogamulizumab	Blood and erythrodermic skin (SS, stage III)	MAVORIC^[25]: PFS 8 vs 3 months; blood response 68% vs 19%	Avoid in autoimmune disease; caution before allo-HCT
Extracorporeal photopheresis	Erythroderma, blood involvement; combine with interferon or bexarotene	Observational series (response in skin common, CR uncommon)	Specialised centres; slow responses; venous access
Bexarotene, methotrexate, interferon	Patch/plaque and itch in advanced MF; add-on to skin-directed therapy	Less active than BV in ALCANZA	Bexarotene: triglycerides, thyroid
Romidepsin, pralatrexate	Tumours, nodes; MF IVB	Phase 2 (romidepsin 34% overall, 57% blood response)	Romidepsin hard for frail patients
Gemcitabine, liposomal doxorubicin	Aggressive or visceral MF/SS	Zinzani 2000^[20] (71%); EORTC 21012^[21] (41%)	Myelosuppression
Rituximab (single agent)	Extensive PCFCL; in-field relapse of PCFCL/PCMZL	Small series; responses often transient	Skin relapse is common
Allogeneic HCT	Advanced MF/SS, fit, best response	CUTALLO^[22]; EBMT^[24] (7-year OS 44%)	Non-relapse mortality about 20%

Table D. Managing problems during treatment

Problem	What to do	Notes
Itch (MF, SS)	Emollients after bathing, topical steroid, antihistamines; doxepin at night, gabapentin or pregabalin; mirtazapine, aprepitant or naltrexone if refractory	Itch tracks stage; a new infection worsens it
Staphylococcus aureus colonisation or infection	Culture crusted or weeping erosions; dilute bleach baths or chlorhexidine plus topical antibiotic; systemic antibiotics (consider MRSA) for real infection	Clearing infection often improves the lymphoma skin signs
Herpes simplex or zoster in erythroderma	Recognise and treat promptly	Risk of disseminated infection
Topical steroid side effects	Watch for atrophy and striae; care near the eyes	Response by 3 months, or change
Phototherapy and RT late effects	Skin cancer and cataract surveillance after PUVA; RT dermatitis, alopecia	Avoid PUVA after many skin cancers
Brentuximab neuropathy	Dose delay, reduction or stop (half needed a change in ALCANZA)	41% still had grade ≤2 neuropathy at long follow-up
HLH (SPTCL, γδ TCL)	Ferritin, triglycerides, fibrinogen in a febrile cytopenic patient; urgent haematology	Main adverse factor in SPTCL

07 Management algorithms

Five decision paths pull together sections 02–06.

Algorithm 1Suspected cutaneous lymphoma: to an entity and a plan

Persistent patches in covered skin, pale patches, erythroderma, nodules, self-healing papules or fat nodules → stop topicals 2–4 weeks → biopsy before steroids or antifungals

Name the entity

Punch ≥4 mm (patches); excision or deep incision (nodules, fat)

IHC: CD3, CD4, CD7, CD8, CD30, ALK, βF1, γδ, CD56; B panel

Epidermotropic CD4 → MF (ISCL score)

CD30 large cells → LyP or C-ALCL

BCL6+ BCL2− → PCFCL; BCL2+ plasma cells → PCMZL; fat rimming CD8 → SPTCL

Stage

MF/SS: % BSA, nodes, flow cytometry + clone (TNMB)

CT/PET-CT if T2b, T3, T4, nodes >1.5 cm, LCT, B2

Other types: PET-CT to exclude systemic lymphoma

ALK nuclear or nodes → systemic ALCL

Before treatment

HTLV-1 if endemic origin, flower cells or high calcium

HBV and HCV before methotrexate

HLH screen if febrile and cytopenic

Pseudolymphoma? Drug, contact, light: clinicopathologic correlation

All: clinicopathologic correlation · repeat biopsies when unsure · a clone or CD30 alone is not a diagnosis.

Algorithm 2Mycosis fungoides, early stage (IA–IIA)

MF confirmed, stage IA–IIA → lesion type, % BSA, folliculotropism, blood class

IA (<10% BSA)

Potent topical steroid

No response by 3 months → mechlorethamine, bexarotene gel, imiquimod or local RT

Many courses over a lifetime

IB–IIA, thin plaques

Steroid, mechlorethamine, NBUVB or PUVA

Clear → slow taper

Relapse → retreat or rotate

PUVA: more CR

Thick plaques, FMF, LCT or B1

Thick, widespread → TSEBT 10–12 Gy

FMF, LCT, B1 → add bexarotene or methotrexate

Adjuvant topical, light or retinoid ≥6 months

Tumour-stage FMF or LCT → Algorithm 3

All: emollients and itch control · no early chemotherapy (no survival gain) · biopsy again if the pace changes.

Algorithm 3Advanced mycosis fungoides and Sézary syndrome

Stage IIB–IV → which compartment dominates: tumours, erythroderma/blood, nodes, viscera?

Tumours (IIB) or LCT

Few → local RT 8–12 Gy + topical

Generalised or LCT → brentuximab vedotin

Then TSEBT; romidepsin, pralatrexate, gemcitabine

ALCANZA

Erythroderma or blood (III, SS IVA)

ECP ± interferon or mogamulizumab

Add bexarotene or interferon

Itch, infection care

MAVORIC

Nodes N3, viscera (IVA2, IVB)

MF: BV, romidepsin or pralatrexate

SS with M1b/LCT: gemcitabine or liposomal doxorubicin

RT 12–24 Gy to symptomatic nodes

Fit, ≤65: allo-HCT at best response

All: multidisciplinary team · maintenance after response · trials · allogeneic HCT is the only cure; no autologous HCT.

Algorithm 4CD30+ disorders and rare cutaneous T-cell lymphomas

CD30+ or cytotoxic infiltrate in the skin → clinical course, size, ALK, phenotype

Lymphomatoid papulosis

Few → observe or topical steroid

Many or face/hands → low-dose methotrexate → PUVA

Lesion >2 cm, not gone in 8 weeks → biopsy

Lifelong follow-up: 15.5% second lymphoma

C-ALCL or ALK+

Solitary/few nodules >2 cm, CD30 ≥75%; ALK+ or wide nodes → systemic ALCL

Localised → excision or ISRT (≤20 Gy)

Multifocal + nodes/symptoms → BV

Asymptomatic multifocal: observe 4–6 weeks

Cytotoxic T-cell types

SPTCL → deep biopsy, HLH screen

γδ, CD8+ AECTCL → aggressive: systemic, transplant

Acral CD8+, CD4+ small/medium → excision, RT or steroid

SPTCL treatment: not yet covered here

All: no multiagent chemotherapy for LyP or C-ALCL · stage fully when ALK+ · HLH is the danger in SPTCL.

Algorithm 5Primary cutaneous follicle centre and marginal zone lymphoma

PCFCL or PCMZL confirmed; PET-CT negative → how many lesions, where, symptomatic?

One RT field

Symptomatic → RT (PCFCL 4 Gy → 24 Gy; PCMZL 24 Gy) or excision

Not troublesome → steroid or observe

ILROG: >4 Gy better local control

Several sites

PCFCL: RT to ≤3 symptomatic sites + steroids

PCMZL: observe if quiet; intralesional steroid, low-dose RT or excision

No chemotherapy

Extensive or relapse

Extensive PCFCL → rituximab

Relapse outside field → RT; inside → excision or rituximab

Spread beyond skin → nodal lymphoma pathway

All: about a third relapse in skin · visits every 3 months for 2 years · no routine scans or blood tests.

08 Survivorship, follow-up & late effects

Figure 14. Follow-up rhythm (Terhorst 2012; UpToDate PCFCL, PCMZL, LyP and MF reviews). Individualise.

Indolent B-cell lymphomas: every 3 months for 2 years, then as needed; in one study all 23 relapses of PCFCL were found by examination, none by blood tests or scans (Terhorst 2012^[41]). LyP: lifelong review because of the second-lymphoma risk.
MF/SS: whole-skin examination with % BSA, nodes and organs, CBC, chemistry, LDH, flow cytometry; repeat imaging only if it was abnormal and would change management.
Late effects and risks: skin cancers and cataracts after PUVA and radiation; steroid atrophy; neuropathy after BV; more second lymphomas after MF/SS and LyP; infections (Staphylococcus, herpes).
Living with it: itch affects sleep and mood (itch scores rise with stage); support, counselling and honest discussion of a chronic, relapsing disease.

Figure 15. Relapse patterns (UpToDate PCFCL, PCMZL, early and advanced MF and SS reviews).

09 Landmark trials

Figure 16. How to read a survival curve (illustrative). Use the absolute gap at a fixed time and overall survival, not the median or PFS alone, when weighing benefit against toxicity and cost.

Figure 17. Landmark results as reported in the UpToDate MF, SS, early-stage MF and PCFCL reviews.

ALCANZA^[5]: BV vs methotrexate or bexarotene in CD30+ MF and C-ALCL → better and longer responses → BV for tumours and LCT.
MAVORIC^[25]: mogamulizumab vs vorinostat → longer PFS, strong blood responses → mogamulizumab for Sézary and erythrodermic MF.
CUTALLO^[22]: allogeneic HCT vs no transplant in advanced CTCL (by donor availability) → longer PFS.
Kaye 1989^[16]: chemotherapy plus electron beam vs topical therapy first → no survival gain → skin-directed first in early MF.
Phan 2019^[10] and Lessin 2013^[9]: the evidence for phototherapy and topical mechlorethamine.
ILROG 2026^[14]: 535 patients; same CR at ≤4 Gy, better local control above 4 Gy.

10 Practice pearls & pitfalls

Persistent patches on the buttocks, groins and trunk that change size and shape: biopsy, after 2–4 weeks off topicals.
Pale patches in a child or young adult that do not behave like leprosy, vitiligo or pityriasis alba: think hypopigmented MF.
Erythroderma in an older adult: flow cytometry (CD4+CD7−/CD26−) and a TCR clone in blood and skin before calling it a drug rash or eczema.
A T-cell clone or CD30+ cells alone do not make a lymphoma: correlate with the clinical picture.
Early MF is a chronic skin disease for most people: skin-directed treatment, not chemotherapy.
New nodules in long-standing plaques: rebiopsy for large cell transformation (BV changes the outlook).
Self-healing papules with large CD30+ cells: lymphomatoid papulosis; never CHOP.
ALK-positive CD30+ skin tumour: stage fully for systemic ALCL.
Fat nodules with fever and cytopenias: SPTCL with HLH until proven otherwise; deep biopsy and ferritin.
Cutaneous B-cell lymphoma on the scalp or trunk: low-dose RT, excision or steroid; chemotherapy is rarely needed.

11 Case vignettes: lines of management

Eight cases from the clinic.

Case 1Hypopigmented MF · teenager

Pale patches treated as leprosy

16 years, boyStage IBCD8+

A 16-year-old student from a village near Nellore has pale, slightly scaly patches on the trunk, buttocks and thighs for 3 years. He received a course of leprosy treatment and antifungal creams; the patches kept spreading. No itch.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Suspect
Hypopigmented patches in covered skin that change in size and do not respond to treatment for leprosy or fungal infection: hypopigmented MF is in the differential.
Biopsy
Creams stopped for 3 weeks; two 4 mm punches: epidermotropic atypical lymphocytes without spongiosis, CD8+, CD7 loss; clonal TCR. ISCL score 5.
Stage
Patches cover about 15% of the skin; no nodes; blood flow cytometry B0: stage IB (T2a N0 M0 B0). Chest X-ray only (CT is for T2b and above).
Treat
NBUVB phototherapy (or topical steroid for small areas); slow taper after clearing. Hypopigmented MF responds well and has a favourable outlook.

Teaching points

Hypopigmented MF is the commonest form in children and darker skin.
Mimics: leprosy, vitiligo, tinea versicolor, pityriasis alba.
Biopsy before more empirical treatment.

See: Sections 02, 03 and 06, Algorithm 2.

Case 2Classic MF · stage IB

Six years of "eczema" on the buttocks

52 years, womanStage IBPatches and thin plaques

A 52-year-old teacher has itchy, scaly, reddish patches on the buttocks, hips and breasts for 6 years, labelled eczema and psoriasis. They cover about 20% of her skin.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Diagnose
Topicals stopped for 2–4 weeks; biopsy from the thickest plaque: epidermotropism, Pautrier microabscesses, CD4+ with CD7 loss; clone present. MF.
Stage
T2b (plaques ≥10%), no nodes, B0: stage IB. CT or PET-CT for T2b; normal.
Treat
Skin-directed: NBUVB or PUVA (or mechlorethamine and potent steroid); clears in months; slow maintenance taper.
Follow
Relapse after stopping maintenance is likely and usually responds again; rotate skin-directed options; systemic agents only if control fails.

Teaching points

Persistent patches in sun-protected skin: biopsy.
Stage IB MF: skin-directed treatment, not chemotherapy.
Most patients with patches never progress.

See: Sections 03, 05 and 06, Algorithm 2.

Case 3MF · tumours with LCT

New nodules in old plaques

64 years, manStage IIBCD30+ LCT

A 64-year-old with plaque MF for 8 years develops several fast-growing nodules on the trunk and thigh, two of them ulcerated.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Rebiopsy
Nodule biopsy: large cells over 25% of the infiltrate (large cell transformation), CD30 in 60%.
Stage
PET-CT: no nodes over 1.5 cm, no viscera; blood B0: stage IIB with LCT. CLIPI: age >60, LCT → 2 points.
Treat
Brentuximab vedotin (ALCANZA) with local RT 8–12 Gy to the ulcerated tumours; watch for neuropathy.
Plan
Fit and 64: refer for allogeneic transplant assessment, to be done at best response; mogamulizumab avoided close to transplant.

Teaching points

A change of pace means a new biopsy.
LCT and generalised tumours: BV.
Allogeneic HCT is the only cure in advanced MF.

See: Sections 04 and 06, Algorithm 3.

Case 4Sézary syndrome

Red, peeling skin all over and unbearable itch

68 years, manStage IVA1B2

A 68-year-old retired engineer has 4 months of red, scaly skin covering almost his whole body, hair loss, thick palms and soles, and itch that stops him sleeping. Treated for a drug rash. Firm nodes in both groins.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Diagnose
Skin biopsy compatible with MF/SS; flow cytometry: 3200/µL CD4+CD7− cells; the same TCR clone in blood and skin. HTLV-1 negative. Sézary syndrome.
Stage
Groin node excision: architecture preserved (N2); PET-CT: no viscera: T4 N2 M0 B2 = stage IVA1.
Treat
Mogamulizumab, or ECP with interferon at a specialised centre; add bexarotene or interferon if needed.
Support
Emollients, bedtime doxepin, gabapentin; skin swabs; bleach baths and topical antibiotic for colonisation; prompt antivirals for herpes.

Teaching points

Erythroderma: flow cytometry before calling it a drug rash.
Sézary: ECP or mogamulizumab first.
Treat itch and infection as seriously as the lymphoma.

See: Sections 03 and 06 (Sézary), Algorithm 3.

Case 5Lymphomatoid papulosis

Crops of papules that crust and heal

34 years, womanLyP type ACD30+

A 34-year-old nurse has crops of red papules on the arms and thighs for 2 years; each crusts in the centre and heals in 4–6 weeks with small scars. She feels well.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Diagnose
Excision of two fresh papules: wedge-shaped mixed infiltrate with large CD30+ cells, ALK negative; TCR clone. LyP type A.
Treat
Few lesions: observation or a super-potent topical steroid. If they become many or appear on her face or hands: low-dose weekly methotrexate (liver and blood monitoring), then PUVA.
Watch
A 3 cm nodule that has not regressed in 8 weeks: excise to look for primary cutaneous ALCL (CD30 on ≥75% of large cells); ALK, HTLV-1 and staging if so.
Follow
Lifelong review: about 15% develop another lymphoma (MF, ALCL). No chemotherapy for LyP itself.

Teaching points

Self-healing CD30+ papules: LyP, not high-grade lymphoma.
Treatment is for symptoms; it does not change the course.
Biopsy persistent nodules over 2 cm.

See: Section 06 (CD30+), Algorithm 4.

Case 6Primary cutaneous ALCL · localised

An ulcerating nodule on the forearm

59 years, manSolitaryCD30 ≥75%

A 59-year-old farmer has a 3 cm red nodule on the forearm that grew over 2 months and ulcerated. Antibiotics for a presumed abscess did not help. No fever or weight loss; a soft 1.5 cm node in the axilla.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Diagnose
Excision biopsy: sheets of large anaplastic cells, CD30 on over 90%, CD4+, ALK and EMA negative; HTLV-1 negative. Primary cutaneous ALCL.
Stage
Axillary node biopsy: reactive (dermatopathic) only. PET-CT: no other disease. Localised skin disease, no nodal involvement.
Treat
The excision has clear margins: observation; adding RT after excision gives no benefit. Had excision not been possible: involved-site RT (low doses, ≤20 Gy, appear enough).
Follow
Skin relapse is common (about 40%) but rarely dangerous: a new nodule is observed first, then excised or irradiated; brentuximab vedotin is kept for multifocal disease with nodes or symptoms, or multiple relapses.

Teaching points

An ulcerating nodule that is not an abscess: biopsy.
Localised C-ALCL: excision or RT, never CHOP.
Biopsy enlarged nodes: most are reactive.

See: Section 06 (CD30+), Algorithm 4.

Case 7PCFCL · scalp

A slowly growing scalp nodule

55 years, manT1aBCL6+ BCL2−

A 55-year-old shop owner has a firm, painless 3 cm pink nodule on the scalp that has grown over a year. No fevers or weight loss.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Diagnose
Excision biopsy: dermal infiltrate sparing the epidermis, large centrocytes; CD20+, BCL6+, BCL2−, MUM1−; FOXP1 and IgM not strong. PCFCL.
Stage
PET-CT: no other disease; CBC and LDH normal: T1a N0 M0.
Treat
RT to the lesion with a 1–1.5 cm margin (response-adapted 4 Gy, escalating to 24 Gy; higher doses give better local control).
Follow
Every 3 months for 2 years with skin examination; no routine scans. A skin relapse outside the field → RT again.

Teaching points

PCFCL is indolent: 5-year survival about 95%.
BCL2, MUM1, FOXP1, IgM strong → leg-type DLBCL instead.
Exclude systemic follicular lymphoma with PET-CT.

See: Sections 05 and 06 (B-cell), Algorithm 5.

Case 8SPTCL · HLH

Fat nodules, fever and falling counts

28 years, womanLupusSPTCL

A 28-year-old with lupus has painless nodules on her thighs and arms for 7 months, called lupus panniculitis. She now has high fevers, a large liver, falling counts and a very high ferritin.

Before you open the answer: what is the likely diagnosis, what must be done before treatment, and what is the plan?

Show the line of management

Suspect
Painless nodules that come and go, then fever and cytopenias: SPTCL with HLH must be excluded (lupus profundus nodules are usually painful).
Biopsy
Deep excisional biopsy with fat: atypical lymphocytes rimming fat cells; CD3+, CD8+, βF1+, cytotoxic; CD4−, CD56−; EBER negative; clonal TCR. SPTCL.
Urgent
HLH work-up (ferritin, triglycerides, fibrinogen) and urgent haematology care; HLH is the main factor for poor outcome (5-year survival 46% vs 91%).
Plan
Treatment is planned with the lymphoma team (SPTCL treatment is not yet covered in this module). Germline HAVCR2 (TIM-3) variants are common in SPTCL with HLH.

Teaching points

Shave biopsies miss SPTCL: biopsy the fat.
Up to 20% of SPTCL patients have autoimmune disease.
Fever and cytopenias in SPTCL: think HLH.

See: Sections 02, 03 and 06 (rare subtypes), Algorithm 4.

12 References & guidelines

NCCN Guidelines: Primary Cutaneous Lymphomas (current version); EORTC/ISCL/USCLC consensus papers listed below; WHO-EORTC 2018 update, WHO Classification 5th edition and the International Consensus Classification (2022).
Cross-checked in October 2026 against 17 UpToDate topic reviews: primary cutaneous anaplastic large cell lymphoma; panniculitis: recognition and diagnosis; classification of primary cutaneous lymphomas; MF diagnosis; variants of MF; staging and prognosis of MF and Sézary syndrome; treatment of early-stage MF; treatment of advanced-stage MF; Sézary syndrome diagnosis; Sézary syndrome treatment; lymphomatoid papulosis; SPTCL; primary cutaneous T-cell lymphomas, rare subtypes; cutaneous T-cell pseudolymphomas; primary cutaneous follicle centre lymphoma; primary cutaneous marginal zone lymphoma; relapsed or refractory PTCL (initial treatment of PTCL and of systemic ALCL, and systemic ALCL diagnosis, also checked). Citations from their reference lists. Indian data web-verified on 17 October 2026. Reviewed and approved by Dr. Haritha Chiramana, 17 October 2026.

Core trials and papers cited on this page (48)

Journal, year, volume and first page (or a PubMed search); each links to PubMed.

Numbered in order of first citation in the text

Cutaneous lymphomas in south India (Khader 2016): Khader A, Manakkad SP, Shaan M, et al. A clinicopathological analysis of primary cutaneous lymphomas: a 6-year observational study at a tertiary care center of south India. Indian J Dermatol 61:608. 2016. doi:10.4103/0019-5154.193665. PubMed India
Hypopigmented MF in Mumbai (KEM): Department of Dermatology, Seth GS Medical College and KEM Hospital, Mumbai. A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides. Indian J Dermatol Venereol Leprol. PubMed India
Defining early MF (Pimpinelli 2005): Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol 2005;53:1053. PubMed Classification, diagnosis, staging and prognosis
ISCL/USCLC/EORTC staging update (Olsen 2022): Olsen EA, Whittaker S, Willemze R, et al. Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC. Blood 2022;140:419. PubMed Classification, diagnosis, staging and prognosis
ALCANZA (Prince 2017): Prince HM, Kim YH, Horwitz SM, et al. Brentuximab vedotin or physician's choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): a randomised, phase 3 trial. Lancet 2017;390:555. PubMed Treatment of mycosis fungoides and Sézary syndrome
CLIPI advanced (Scarisbrick 2025): Scarisbrick JJ, Quaglino P, Whittaker S, et al. A new prognostic index (CLIPI) for advanced cutaneous lymphoma. Blood 2025;146:1687. PubMed Classification, diagnosis, staging and prognosis
Early-stage CLIPi (Benton 2013): Benton EC, Crichton S, Talpur R, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sézary syndrome. Eur J Cancer 2013;49:2859. PubMed Classification, diagnosis, staging and prognosis
Topical steroids in MF (Zackheim 1998): Zackheim HS, Kashani-Sabet M, Amin S. Topical corticosteroids for mycosis fungoides: experience in 79 patients. Arch Dermatol 1998;134:949. PubMed Treatment of mycosis fungoides and Sézary syndrome
Mechlorethamine gel trial (Lessin 2013): Lessin SR, Duvic M, Guitart J, et al. Topical chemotherapy in cutaneous T-cell lymphoma: a randomized trial of mechlorethamine 0.02% gel in mycosis fungoides. JAMA Dermatol 2013;149:25. PubMed Treatment of mycosis fungoides and Sézary syndrome
NBUVB vs PUVA meta-analysis (Phan 2019): Phan K, Ramachandran V, Fassihi H, Sebaratnam DF. Comparison of narrowband UV-B with psoralen-UV-A phototherapy for early-stage mycosis fungoides: systematic review and meta-analysis. JAMA Dermatol 2019;155:335. PubMed Treatment of mycosis fungoides and Sézary syndrome
Single-fraction RT in CTCL (Thomas 2013): Thomas TO, Agrawal P, Guitart J, et al. Outcome of patients treated with a single-fraction dose of palliative radiation for cutaneous T-cell lymphoma. Int J Radiat Oncol Biol Phys 2013;85:747. PubMed Treatment of mycosis fungoides and Sézary syndrome
Low-dose TSEBT pooled (Hoppe 2015): Hoppe RT, Harrison C, Tavallaee M, et al. Low-dose total skin electron beam therapy in mycosis fungoides: pooled analysis of 3 phase II trials. J Am Acad Dermatol 2015;72:286. PubMed Treatment of mycosis fungoides and Sézary syndrome
Low-dose 10 Gy TSEBT (Kamstrup 2015): Kamstrup MR, Gniadecki R, Iversen L, et al. Low-dose (10-Gy) total skin electron beam therapy for cutaneous T-cell lymphoma: an open clinical study and pooled data analysis. Int J Radiat Oncol Biol Phys 2015;92:138. PubMed Treatment of mycosis fungoides and Sézary syndrome
ILROG RT for indolent PCBCL (Oertel 2026): Oertel M, Dabaja B, Görlich D, et al. Radiotherapy for indolent primary cutaneous B-cell lymphoma: an international multicenter ILROG analysis. Blood 2026;147:3051. PubMed Cutaneous B-cell lymphomas and follow-up
ILROG RT field and dose (Specht 2015): Specht L, Dabaja B, Illidge T, et al. Modern radiation therapy for primary cutaneous lymphomas: field and dose guidelines from ILROG. Int J Radiat Oncol Biol Phys 2015;92:32. PubMed Cutaneous B-cell lymphomas and follow-up
Early aggressive therapy trial (Kaye 1989): Kaye FJ, Bunn PA Jr, Steinberg SM, et al. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med 1989;321:1784. PubMed Treatment of mycosis fungoides and Sézary syndrome
Folliculotropic MF (Hodak 2016): Hodak E, Amitay-Laish I, Atzmony L, et al. New insights into folliculotropic mycosis fungoides: a single-center experience. J Am Acad Dermatol 2016;75:347. PubMed Classification, diagnosis, staging and prognosis
Folliculotropic MF treatment (van Santen 2017): van Santen S, van Doorn R, Neelis KJ, et al. Recommendations for treatment in folliculotropic mycosis fungoides: report of the Dutch Cutaneous Lymphoma Group. Br J Dermatol 2017;177:223. PubMed Treatment of mycosis fungoides and Sézary syndrome
ALCANZA final (Horwitz 2021): Horwitz SM, Scarisbrick JJ, Dummer R, et al. Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data. Blood Adv 2021;5:5098. PubMed Treatment of mycosis fungoides and Sézary syndrome
Gemcitabine in CTCL (Zinzani 2000): Zinzani PL, Baliva G, Magagnoli M, et al. Gemcitabine treatment in pretreated cutaneous T-cell lymphoma: experience in 44 patients. J Clin Oncol 2000;18:2603. PubMed Treatment of mycosis fungoides and Sézary syndrome
Liposomal doxorubicin EORTC 21012 (Dummer 2012): Dummer R, Quaglino P, Becker JC, et al. Pegylated liposomal doxorubicin in stage IIB, IVA or IVB mycosis fungoides: final results from EORTC 21012. J Clin Oncol 2012;30:4091. PubMed Treatment of mycosis fungoides and Sézary syndrome
CUTALLO (de Masson 2023): de Masson A, Beylot-Barry M, Ram-Wolff C, et al. Allogeneic transplantation in advanced cutaneous T-cell lymphomas (CUTALLO): a propensity score matched controlled prospective study. Lancet 2023;401:1941. PubMed Treatment of mycosis fungoides and Sézary syndrome
CIBMTR allo-HCT in MF/SS (Lechowicz 2014): Lechowicz MJ, Lazarus HM, Carreras J, et al. Allogeneic hematopoietic cell transplantation for mycosis fungoides and Sézary syndrome. Bone Marrow Transplant 2014;49:1360. PubMed Treatment of mycosis fungoides and Sézary syndrome
EBMT allo-HCT long-term (Duarte 2014): Duarte RF, Boumendil A, Onida F, et al. Long-term outcome of allogeneic HCT for mycosis fungoides and Sézary syndrome: EBMT lymphoma working party. J Clin Oncol 2014;32:3347. PubMed Treatment of mycosis fungoides and Sézary syndrome
MAVORIC (Kim 2018): Kim YH, Bagot M, Pinter-Brown L, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): a randomised, controlled phase 3 trial. Lancet Oncol 2018;19:1192. PubMed Treatment of mycosis fungoides and Sézary syndrome
CD30+ LPD consensus (Kempf 2011): Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders. Blood 2011;118:4024. PubMed CD30+ disorders and rare T-cell subtypes
LyP associated malignancies (Melchers 2020): Melchers RC, Willemze R, Bekkenk MW, et al. Frequency and prognosis of associated malignancies in 504 patients with lymphomatoid papulosis. J Eur Acad Dermatol Venereol 2020;34:260. PubMed CD30+ disorders and rare T-cell subtypes
CD30+ LPD Dutch series (Bekkenk 2000): Bekkenk MW, Geelen FA, van Voorst Vader PC, et al. Primary and secondary cutaneous CD30+ lymphoproliferative disorders: long-term follow-up of 219 patients. Blood 2000;95:3653. PubMed CD30+ disorders and rare T-cell subtypes
ILROG RT for PC-ALCL (Million 2016): Million L, Yi EJ, Wu F, et al. Radiation therapy for primary cutaneous anaplastic large cell lymphoma: an International Lymphoma Radiation Oncology Group multi-institutional experience. Int J Radiat Oncol Biol Phys 2016;95:1454. PubMed CD30+ disorders and rare T-cell subtypes
Low-dose RT for PC-ALCL (Smith 2017): Smith GL, Duvic M, Yehia ZA, et al. Effectiveness of low-dose radiation for primary cutaneous anaplastic large cell lymphoma. Adv Radiat Oncol 2017;2:363. PubMed CD30+ disorders and rare T-cell subtypes
PC-ALCL Dutch multifocal (Melchers 2018): Melchers RC, Willemze R, Bekkenk MW, et al. Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group. Br J Dermatol 2018;179:724. PubMed CD30+ disorders and rare T-cell subtypes
PC-ALCL in British Columbia (Hapgood 2017): Hapgood G, Pickles T, Sehn LH, et al. Outcome of primary cutaneous anaplastic large cell lymphoma: a 20-year British Columbia Cancer Agency experience. Br J Haematol 2017;176:234. PubMed CD30+ disorders and rare T-cell subtypes
PC-ALCL in SEER (Sarfraz 2021): Sarfraz H, Gentille C, Ensor J, et al. Primary cutaneous anaplastic large-cell lymphoma: a review of the SEER database from 2005 to 2016. Clin Exp Dermatol 2021;46:1420. PubMed CD30+ disorders and rare T-cell subtypes
PC-ALCL prognosis in Japan (Miyagaki 2023): Miyagaki T, Inoue N, Kamijo H, et al. Prognostic factors for primary cutaneous anaplastic large-cell lymphoma: a multicentre retrospective study from Japan. Br J Dermatol 2023;189:612. PubMed CD30+ disorders and rare T-cell subtypes
SPTCL EORTC study (Willemze 2008): Willemze R, Jansen PM, Cerroni L, et al. Subcutaneous panniculitis-like T-cell lymphoma: an EORTC Cutaneous Lymphoma Group study of 83 cases. Blood 2008;111:838. PubMed CD30+ disorders and rare T-cell subtypes
HAVCR2 in SPTCL (Gayden 2018): Gayden T, Sepulveda FE, Khuong-Quang DA, et al. Germline HAVCR2 mutations altering TIM-3 characterize SPTCL with hemophagocytic lymphohistiocytic syndrome. Nat Genet 2018;50:1650. PubMed CD30+ disorders and rare T-cell subtypes
Gamma-delta CTCL survival (Toro 2003): Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood 2003;101:3407. PubMed CD30+ disorders and rare T-cell subtypes
CD8+ AECTCL (Berti 1999): Berti E, Tomasini D, Vermeer MH, et al. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas: a distinct entity with aggressive behavior. Am J Pathol 1999;155:483. PubMed CD30+ disorders and rare T-cell subtypes
Acral CD8+ LPD (Kempf 2022): Kempf W, Petrella T, Willemze R, et al. Primary cutaneous acral CD8+ T-cell lymphoma and other dermal CD8+ cutaneous lymphoproliferations: EORTC workshop. Br J Dermatol 2022;186:887. PubMed CD30+ disorders and rare T-cell subtypes
Cutaneous B-cell lymphoma consensus (Senff 2008): Senff NJ, Noordijk EM, Kim YH, et al. EORTC and ISCL consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008;112:1600. PubMed Cutaneous B-cell lymphomas and follow-up
Follow-up of indolent cutaneous lymphoma (Terhorst 2012): Terhorst D, Mestel DS, Humme D, et al. Evaluation of different methods in the follow-up of patients with indolent types of primary cutaneous lymphomas. Br J Dermatol 2012;166:1295. PubMed Cutaneous B-cell lymphomas and follow-up

Further reading (not cited in the text above)

WHO-EORTC classification (Willemze 2005): Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768. PubMed Classification, diagnosis, staging and prognosis
WHO-EORTC 2018 update (Willemze 2019): Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019;133:1703. PubMed Classification, diagnosis, staging and prognosis
ISCL/EORTC staging revision (Olsen 2007): Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome (ISCL/EORTC). Blood 2007;110:1713. PubMed Classification, diagnosis, staging and prognosis
TNM for non-MF cutaneous lymphoma (Kim 2007): Kim YH, Willemze R, Pimpinelli N, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary syndrome. Blood 2007;110:479. PubMed Classification, diagnosis, staging and prognosis
MF in a multi-ethnic Asian cohort (Lim 2019): Lim HLJ, Tan EST, Tee SI, et al. Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi-ethnic Asian cohort: a 12-year review. J Eur Acad Dermatol Venereol 2019;33:1513. PubMed Classification, diagnosis, staging and prognosis
Sézary syndrome at Mayo (Kubica 2012): Kubica AW, Davis MD, Weaver AL, et al. Sézary syndrome: a study of 176 patients at Mayo Clinic. J Am Acad Dermatol 2012;67:1189. PubMed Classification, diagnosis, staging and prognosis
4 Gy RT for indolent B-cell lymphoma (Imber 2021): Imber BS, Chau KW, Lee J, et al. Excellent response to very-low-dose radiation (4 Gy) for indolent B-cell lymphomas. Blood Adv 2021;5:4185. PubMed Cutaneous B-cell lymphomas and follow-up

How to cite this page

VancouverChiramana H. Cutaneous Lymphomas: a mini review for clinicians. Oncology Knowledge Hub [Internet]. Nellore (India): drharitha.co.in; 2026 [updated 2026 Oct 17; cited year month day]. Available from: https://drharitha.co.in/professionals/cutaneous-lymphomas

APA 7Chiramana, H. (2026, October 17). Cutaneous Lymphomas: A mini review for clinicians. Oncology Knowledge Hub. Retrieved Month Day, Year, from https://drharitha.co.in/professionals/cutaneous-lymphomas

Free to use and share for education with attribution; please do not alter or use commercially (CC BY-NC-ND 4.0). Replace the italic parts with the date you accessed the page.

Educational summary for health professionals, written in our own words from current guidelines and primary trials. No drug doses are given. Hodgkin, aggressive B-cell, indolent B-cell and T-cell lymphomas are parts 1–4; primary cutaneous diffuse large B-cell lymphoma, leg type, is covered in the aggressive B-cell module. Verify current guidelines, Indian regulatory approvals and individual patient factors before treatment decisions.

Written by Dr. Haritha ChiramanaSenior Medical Oncologist & Hemato-Oncologist · MD, DM (Medical Oncology) · C.R. Reddy Cancer Center, Nellore, Andhra Pradesh, India

Medically reviewed by Dr. Haritha Chiramana on 17 October 2026. This page is general education and cannot replace an individual consultation.