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Dermatology135 papers

Cutaneous T-cell lymphoma

Last edited: 4/14/2026

Overview

Cutaneous T-cell lymphoma (CTCL) encompasses a group of non-Hodgkin lymphomas primarily affecting the skin, with mycosis fungoides and Sézary syndrome being the most common subtypes 122.

Diagnosis

  • Clinical examination focusing on skin lesions, often symmetrically distributed 1.
  • Biopsy with histopathological examination to confirm malignant T-cell infiltration 122.
  • Immunohistochemistry to identify specific T-cell markers (e.g., CD4, CD30) 134.
  • Circulating Sézary cells detected in peripheral blood for Sézary syndrome 34.
  • Genetic studies, including TCR sequencing, for deeper characterization 14.

    • Management

  • First-line treatments:
    • - Topical therapies (e.g., corticosteroids, nitrogen mustard) for early-stage mycosis fungoides 13. - Systemic therapies: interferon (e.g., IFN-α) for maintenance and control 27.
  • Adjunctive treatments:
    • - Extracorporeal photopheresis (ECP) for advanced or refractory cases 71012. - Mogamulizumab, a CCR4-targeted monoclonal antibody, for relapsed/refractory CTCL 2. - Non-pegylated liposomal doxorubicin as an alternative systemic agent 17. - Vorinostat, a histone deacetylase inhibitor, for systemic involvement 19.

    Special Populations

  • Elderly: ECP may be particularly beneficial due to its immunomodulatory effects and lower toxicity profile 710.
  • Comorbidities: Careful consideration of QT interval prolongation with vorinostat use in patients with cardiac risk factors 19.

    • Key Recommendations

  • Biopsy and histopathological examination are essential for diagnosing CTCL 122 (Evidence: Strong).
  • Extracorporeal photopheresis (ECP) should be considered for advanced or refractory CTCL cases 71012 (Evidence: Moderate).
  • Monitor for immune-related adverse events when using mogamulizumab, especially in real-world settings 2 (Evidence: Moderate).
  • Avoid unmasking or progression of CTCL in patients on dupilumab; monitor closely 45 (Evidence: Weak).
  • Consider genetic and TCR sequencing for deeper disease characterization and potential personalized treatment approaches 14 (Evidence: Expert opinion).

    • References

    1 Swallow MA, Edelson R, Girardi M. A Yale Dermatology perspective on cutaneous T cell lymphoma: Historic reflection to emerging therapies. Clinics in dermatology 2025. link 2 Miyata K, Izawa-Ishizawa Y, Niimura T, Yoshioka T, Hyodo M, Itokazu S et al.. Pharmacovigilance study for the identification of mogamulizumab-induced immune-related adverse events using a real-world database. The oncologist 2025. link 3 Ødum AF, Geisler C. Vitamin D in Cutaneous T-Cell Lymphoma. Cells 2024. link 4 Park A, Wong L, Lang A, Kraus C, Anderson N, Elsensohn A. Cutaneous T-cell lymphoma following dupilumab use: a systematic review. International journal of dermatology 2023. link 5 Schaefer L, Comfere N, Sokumbi O. Development of Cutaneous T-Cell Lymphoma Following Biologic Treatment: A Systematic Review. American journal of clinical dermatology 2023. link 6 Dominguez M, Finnell JT. Unsupervised SoftOtsuNet Augmentation for Clinical Dermatology Image Classifiers. AMIA ... Annual Symposium proceedings. AMIA Symposium 2023. link 7 Lagacé F, Netchiporouk E, Turchin I, Gulliver W, Dutz J, Kirchhof MG et al.. Extracorporeal Photopheresis and Its Use in Clinical Dermatology in Canada. Skin therapy letter 2022. link 8 Pandey S, Dolan A, Norton-Westbrook M, Desai AV. "Knock Me Out": The Challenges of Managing Pain Due to Cutaneous T Cell Lymphomas: A Case Report. Journal of palliative medicine 2022. link 9 Motamedi M, Xiao MZX, Iyer A, Gniadecki R. Patterns of Gene Expression in Cutaneous T-Cell Lymphoma: Systematic Review of Transcriptomic Studies in Mycosis Fungoides. Cells 2021. link 10 Knobler R, Arenberger P, Arun A, Assaf C, Bagot M, Berlin G et al.. European dermatology forum: Updated guidelines on the use of extracorporeal photopheresis 2020 - Part 2. Journal of the European Academy of Dermatology and Venereology : JEADV 2021. link 11 Shamim H, Riemer C, Weenig R, Sokumbi O, Sciallis G, McEvoy M et al.. Acneiform Presentations of Folliculotropic Mycosis Fungoides. The American Journal of dermatopathology 2021. link 12 Knobler R, Arenberger P, Arun A, Assaf C, Bagot M, Berlin G et al.. European dermatology forum - updated guidelines on the use of extracorporeal photopheresis 2020 - part 1. Journal of the European Academy of Dermatology and Venereology : JEADV 2020. link 13 Duffy R, Jennings T, Kartan S, Song A, Shi W, Porcu P et al.. Special Considerations in the Treatment of Mycosis Fungoides. American journal of clinical dermatology 2019. link 14 Matos TR, de Rie MA, Teunissen MBM. Research Techniques Made Simple: High-Throughput Sequencing of the T-Cell Receptor. The Journal of investigative dermatology 2017. link 15 Magro CM, Olson LC, Fulmer CG. CD30+ T cell enriched primary cutaneous CD4+ small/medium sized pleomorphic T cell lymphoma: A distinct variant of indolent CD4+ T cell lymphoproliferative disease. Annals of diagnostic pathology 2017. link 16 Vancurova I. DMF: a promising therapeutic option in CTCL. Blood 2016. link 17 Assaf C, Becker JC, Beyer M, Cozzio A, Dippel E, Klemke CD et al.. Treatment of advanced cutaneous T-cell lymphomas with non-pegylated liposomal doxorubicin--consensus of the lymphoma group of the Working Group Dermatologic Oncology. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2013. link 18 Slade KL, Carreau NA, Heald P. Ethics of clinical trials in dermatology. Clinics in dermatology 2012. link 19 Lynch DR, Washam JB, Newby LK. QT interval prolongation and torsades de pointes in a patient undergoing treatment with vorinostat: a case report and review of the literature. Cardiology journal 2012. link 20 Miyagaki T, Sugaya M. Erythrodermic cutaneous T-cell lymphoma: how to differentiate this rare disease from atopic dermatitis. Journal of dermatological science 2011. link 21 Zic JA. Controversies in the management of the cutaneous T cell lymphomas. Dermatologic therapy 2009. link 22 Willemze R, Meijer CJ. Classification of cutaneous T-cell lymphoma: from Alibert to WHO-EORTC. Journal of cutaneous pathology 2006. link 23 Batton B, Amanullah A, Main C, Fivenson D, Jamil S. Cutaneous T-cell lymphoma in a 21-year-old male with Wolf-Hirschhorn syndrome. American journal of medical genetics. Part A 2004. link 24 Musse L. Cutaneous T-cell lymphoma. Dermatology nursing 2002. link 25 Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L et al.. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. Journal of the American Academy of Dermatology 2002. link 26 Duvic M. Treatment of cutaneous T-cell lymphoma from a dermatologist's perspective. Clinical lymphoma 2000. link 27 Stadler R. Interferons in dermatology. Present-day standard. Dermatologic clinics 1998. link70020-6) 28 Papadavid E, Yu RC, Bunker C, Scoones D, Chu AC. Tumour progression in a patient with granulomatous mycosis fungoides. The British journal of dermatology 1996. link 29 Camisa C, Aulisio A. Pustular mycosis fungoides. Cutis 1994. link 30 Zemtsov A, Camisa C. Treatment of primary cutaneous B cell lymphoma with local radiotherapy. Cutis 1990. link 31 Savage L, Myerly J, Baynes N. Mycosis fungoides: a case study and nursing implications. Dermatology nursing 1990. link 32 Dalziel KL, Telfer NR, Dawber RP. Nail dystrophy in cutaneous T-cell lymphoma. The British journal of dermatology 1989. link 33 Jaworsky C, Murphy GF. Special techniques in dermatology. Archives of dermatology 1989. link 34 Berger R, Bernheim A. Cytogenetic studies of Sézary cells. Cancer genetics and cytogenetics 1987. link90263-9) 35 Alcalay J, David M, Shohat B, Sandbank M. Generalized vitiligo following Sézary syndrome. The British journal of dermatology 1987. link 36 Volc-Platzer B, Groh V, Wolff K. Differential expression of class II alloantigens by keratinocytes in disease. The Journal of investigative dermatology 1987. link 37 Sauder DN, Bergfeld WF. Sézary syndrome. Cutis 1979. link 38 Stoltz JF, Janot C, Humbert JC, Beurey J. Electrophoretic mobility of Sézary syndrome lymphoid cells. Biomedicine / [publiee pour l'A.A.I.C.I.G.] 1979. link

    Original source

    1. [1]
      A Yale Dermatology perspective on cutaneous T cell lymphoma: Historic reflection to emerging therapies. — Swallow MA, Edelson R, Girardi M Clinics in dermatology (2025)
    2. [2]
      Pharmacovigilance study for the identification of mogamulizumab-induced immune-related adverse events using a real-world database. — Miyata K, Izawa-Ishizawa Y, Niimura T, Yoshioka T, Hyodo M, Itokazu S et al. The oncologist (2025)
    3. [3]
      Vitamin D in Cutaneous T-Cell Lymphoma. — Ødum AF, Geisler C Cells (2024)
    4. [4]
      Cutaneous T-cell lymphoma following dupilumab use: a systematic review. — Park A, Wong L, Lang A, Kraus C, Anderson N, Elsensohn A International journal of dermatology (2023)
    5. [5]
      Development of Cutaneous T-Cell Lymphoma Following Biologic Treatment: A Systematic Review. — Schaefer L, Comfere N, Sokumbi O American journal of clinical dermatology (2023)
    6. [6]
      Unsupervised SoftOtsuNet Augmentation for Clinical Dermatology Image Classifiers. — Dominguez M, Finnell JT AMIA ... Annual Symposium proceedings. AMIA Symposium (2023)
    7. [7]
      Extracorporeal Photopheresis and Its Use in Clinical Dermatology in Canada. — Lagacé F, Netchiporouk E, Turchin I, Gulliver W, Dutz J, Kirchhof MG et al. Skin therapy letter (2022)
    8. [8]
      "Knock Me Out": The Challenges of Managing Pain Due to Cutaneous T Cell Lymphomas: A Case Report. — Pandey S, Dolan A, Norton-Westbrook M, Desai AV Journal of palliative medicine (2022)
    9. [9]
      Patterns of Gene Expression in Cutaneous T-Cell Lymphoma: Systematic Review of Transcriptomic Studies in Mycosis Fungoides. — Motamedi M, Xiao MZX, Iyer A, Gniadecki R Cells (2021)
    10. [10]
      European dermatology forum: Updated guidelines on the use of extracorporeal photopheresis 2020 - Part 2. — Knobler R, Arenberger P, Arun A, Assaf C, Bagot M, Berlin G et al. Journal of the European Academy of Dermatology and Venereology : JEADV (2021)
    11. [11]
      Acneiform Presentations of Folliculotropic Mycosis Fungoides. — Shamim H, Riemer C, Weenig R, Sokumbi O, Sciallis G, McEvoy M et al. The American Journal of dermatopathology (2021)
    12. [12]
      European dermatology forum - updated guidelines on the use of extracorporeal photopheresis 2020 - part 1. — Knobler R, Arenberger P, Arun A, Assaf C, Bagot M, Berlin G et al. Journal of the European Academy of Dermatology and Venereology : JEADV (2020)
    13. [13]
      Special Considerations in the Treatment of Mycosis Fungoides. — Duffy R, Jennings T, Kartan S, Song A, Shi W, Porcu P et al. American journal of clinical dermatology (2019)
    14. [14]
      Research Techniques Made Simple: High-Throughput Sequencing of the T-Cell Receptor. — Matos TR, de Rie MA, Teunissen MBM The Journal of investigative dermatology (2017)
    15. [15]
      CD30+ T cell enriched primary cutaneous CD4+ small/medium sized pleomorphic T cell lymphoma: A distinct variant of indolent CD4+ T cell lymphoproliferative disease. — Magro CM, Olson LC, Fulmer CG Annals of diagnostic pathology (2017)
    16. [16]
      DMF: a promising therapeutic option in CTCL. — Vancurova I Blood (2016)
    17. [17]
      Treatment of advanced cutaneous T-cell lymphomas with non-pegylated liposomal doxorubicin--consensus of the lymphoma group of the Working Group Dermatologic Oncology. — Assaf C, Becker JC, Beyer M, Cozzio A, Dippel E, Klemke CD et al. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG (2013)
    18. [18]
      Ethics of clinical trials in dermatology. — Slade KL, Carreau NA, Heald P Clinics in dermatology (2012)
    19. [19]
      QT interval prolongation and torsades de pointes in a patient undergoing treatment with vorinostat: a case report and review of the literature. — Lynch DR, Washam JB, Newby LK Cardiology journal (2012)
    20. [20]
      Erythrodermic cutaneous T-cell lymphoma: how to differentiate this rare disease from atopic dermatitis. — Miyagaki T, Sugaya M Journal of dermatological science (2011)
    21. [21]
      Controversies in the management of the cutaneous T cell lymphomas. — Zic JA Dermatologic therapy (2009)
    22. [22]
      Classification of cutaneous T-cell lymphoma: from Alibert to WHO-EORTC. — Willemze R, Meijer CJ Journal of cutaneous pathology (2006)
    23. [23]
      Cutaneous T-cell lymphoma in a 21-year-old male with Wolf-Hirschhorn syndrome. — Batton B, Amanullah A, Main C, Fivenson D, Jamil S American journal of medical genetics. Part A (2004)
    24. [24]
      Cutaneous T-cell lymphoma. — Musse L Dermatology nursing (2002)
    25. [25]
      Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. — Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L et al. Journal of the American Academy of Dermatology (2002)
    26. [26]
      Treatment of cutaneous T-cell lymphoma from a dermatologist's perspective. — Duvic M Clinical lymphoma (2000)
    27. [27]
      Interferons in dermatology. Present-day standard. — Stadler R Dermatologic clinics (1998)
    28. [28]
      Tumour progression in a patient with granulomatous mycosis fungoides. — Papadavid E, Yu RC, Bunker C, Scoones D, Chu AC The British journal of dermatology (1996)
    29. [29]
      Pustular mycosis fungoides. — Camisa C, Aulisio A Cutis (1994)
    30. [30]
      Treatment of primary cutaneous B cell lymphoma with local radiotherapy. — Zemtsov A, Camisa C Cutis (1990)
    31. [31]
      Mycosis fungoides: a case study and nursing implications. — Savage L, Myerly J, Baynes N Dermatology nursing (1990)
    32. [32]
      Nail dystrophy in cutaneous T-cell lymphoma. — Dalziel KL, Telfer NR, Dawber RP The British journal of dermatology (1989)
    33. [33]
      Special techniques in dermatology. — Jaworsky C, Murphy GF Archives of dermatology (1989)
    34. [34]
      Cytogenetic studies of Sézary cells. — Berger R, Bernheim A Cancer genetics and cytogenetics (1987)
    35. [35]
      Generalized vitiligo following Sézary syndrome. — Alcalay J, David M, Shohat B, Sandbank M The British journal of dermatology (1987)
    36. [36]
      Differential expression of class II alloantigens by keratinocytes in disease. — Volc-Platzer B, Groh V, Wolff K The Journal of investigative dermatology (1987)
    37. [37]
      Sézary syndrome. — Sauder DN, Bergfeld WF Cutis (1979)
    38. [38]
      Electrophoretic mobility of Sézary syndrome lymphoid cells. — Stoltz JF, Janot C, Humbert JC, Beurey J Biomedicine / [publiee pour l'A.A.I.C.I.G.] (1979)
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