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Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge

Received: 17 March 2022     Accepted: 11 June 2022     Published: 29 September 2022
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Abstract

Mycosis Fongoïde is a rare T cell lymphoma. The mean age of onset is about sixty year old, mostly in masculin population. The clinical presentation is dominated by cutaneous signs. We report the case of a 78 year old patient, with a history of erythroderma with pruritus non ameliorated by corticosteroid topics, who was readmitted four years later for osteomyelopathy. At physical examination, he had bilateral axillary lymph nodes. We also noted dry generalized erythroderma with no interval of healthy skin and at the neurologic exam, there was ataxia on walking and a bilateral pyramidal syndrome majorated on the right. The blood count showed hyperleukocytosis with hyperlymphocytosis and chronic hypereosinophilia. The blood smear showed Sezary cells of the order of 2000. The CT scan has confirmed the presence of axillary adenopathies, left breast nodules not found on breast ultrasound, and bilateral adenomegaly of the external iliac and inguinal chains. The first cutaneous biopsy was not conclusive. Axillary node biopsy, 6th skin biopsy and Immunophenotyping of circulating CD4+ T lymphoctes allowed to retain Mycosis fungoides. He was treated with Methotrexate for a year with improvement. Mycosis Fungoides is a rare disease with diagnostic and therapeutic difficulties due to the non contribution of confirmatory examinations wich may delay the diagnostic and with a possibility of atypical presentation.

Published in International Journal of Clinical Dermatology (Volume 5, Issue 2)
DOI 10.11648/j.ijcd.20220502.11
Page(s) 11-14
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2022. Published by Science Publishing Group

Keywords

Cutaneous Lymphoma, Epidermotropic Lymphoma, Mycosis Fungoides, Sezary's Syndrome

References
[1] M. Beylot-Barrya, O. Dereureb, B. Vergierc, S. Bareted, L. Larochee, L. Machetf, M.-H. Delfau-Larueg, M. D’Incanh, F. Grangei, N. Ortonnej, J.-P. Merliok, m, M. Bagotl, pour le Groupe francaisd’étude des lymphomes cutanés (GFELC), Management of cutaneous T-cell lymphomas: Recommendations of the French Cutaneous Lymphoma Group. Ann Dermatol Venereol. 2010 Oct; 137 (10): 611-21.
[2] S. Jabran-Maanaoui, P. Chauvet, M. Gillarda, B. Carpentier, L. Pascal, J.-F. Quinchonc, P. Modianoa. Atypical Sézary syndrome in a young subject. Ann Dermatol Venereol. 2020 May; 147 (5): 355-360.
[3] L. Guilloton, A. Drouet, J. L. Estival, G. Saint Pierre, M. Dupin, C. Ribot. Mycosis fongoïde avec transformation en un lymphome cutané pléiomorphe et localisation secondaire cérébrale. Rev Med Interne. 2001 Dec; 22 (12): 1244-7.
[4] Azzouzi S, Draou N, El Attar H, Benchikhi H, Lakhdar H, Iraqi A. Value of histological criteria in the diagnosis of fungoidal mycosis at the non-tumoral stage. Annales de Pathologie Volume 24, Supplement 1, November 2004, Pages 139-140.
[5] Noel C. Mcfadden. Mycosis Fungoides Unsolved Problems of Diagnosis and Choice of Therapy. Int J Dermatol. 1984 Oct; 23 (8): 523-30.
[6] Franz Trautinger, Johanna Eder, Chalid Assaf, Martine Bagot, Antonio Cozzio, Reinhard Dummer, Robert Gniadecki, Claus-Detlev Klemke, Pablo L. Ortiz-Romero, Evangelia Papadavid, Nicola Pimpinelli, Pietro Quaglino, Annamari Ranki, Julia Scarisbrick, Rudolf Stadler, Liisa Va¨keva¨, Maarten H. Vermeer, Sean Whittaker, Rein Willemze, Robert Knobler, European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Se ́zary syndrome Update 2017. Eur J Cancer. 2017 May; 77: 57-74.
[7] Larocca C, Kupper T. Mycosis Fungoides and Sézary Syndrome: An Update. Hematol Oncol Clin North Am. 2019 Feb; 33 (1): 103-120.
[8] Lovgren ML, Scarisbrick JJ. Update on skin directed therapies in mycosis fungoides. Chin Clin Oncol. 2019 Feb; 8 (1): 7.
[9] Duffy R, Jennings T, Kartan S, Song A, Shi W, Porcu P, Alpdogan O, Sahu J. Special Considerations in the Treatment of Mycosis Fungoides. Am J Clin Dermatol. 2019 Aug; 20 (4): 571-578.
[10] Kamijo H, Miyagaki T. Mycosis Fungoides and Sézary Syndrome: Updates and Review of Current Therapy. Curr Treat Options Oncol. 2021 Jan 7; 22 (2): 10.
[11] Ohtsuka. M. Current Treatment of Mycosis Fungoides]. Gan To Kagaku Ryoho. 2021 Apr; 48 (4): 508-512.
[12] Willemze R. Mycosis fungoides variants-clinicopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018 Mar; 37 (1): 11-17.
[13] Suzanne O. Arulogun, H. Miles Prince, Jonathan Ng, Stephen Lade, Gail F. Ryan, Odette Blewitt, and Christopher McCormack. Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation. Blood. 2008 Oct 15; 112 (8): 3082-7.
[14] F. GRANGE. Prognosis of primitive cutaneous T lymphomas. Annales de dermatologie et de vénéréologie 2002; Vol 129: pp 30-40.
[15] Lansigan F, Horwitz SM, Pinter-Brown LC, Carson KR, Shustov AR, Rosen ST, Pro B, Hsi ED, Federico M, Gisselbrecht C, Schwartz M, Bellm LA, Acosta M, Foss FM. Outcomes of Patients with Transformed Mycosis Fungoides: Analysis from a Prospective Multicenter US Cohort Study. Clin Lymphoma Myeloma Leuk. 2020 Nov; 20 (11): 744-748.
Cite This Article
  • APA Style

    Roua Mankai, Fatma Daoud, Imene Rachdi, Fatma Khanchel, Mehdi Somai, et al. (2022). Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge. International Journal of Clinical Dermatology, 5(2), 11-14. https://doi.org/10.11648/j.ijcd.20220502.11

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    ACS Style

    Roua Mankai; Fatma Daoud; Imene Rachdi; Fatma Khanchel; Mehdi Somai, et al. Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge. Int. J. Clin. Dermatol. 2022, 5(2), 11-14. doi: 10.11648/j.ijcd.20220502.11

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    AMA Style

    Roua Mankai, Fatma Daoud, Imene Rachdi, Fatma Khanchel, Mehdi Somai, et al. Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge. Int J Clin Dermatol. 2022;5(2):11-14. doi: 10.11648/j.ijcd.20220502.11

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  • @article{10.11648/j.ijcd.20220502.11,
      author = {Roua Mankai and Fatma Daoud and Imene Rachdi and Fatma Khanchel and Mehdi Somai and Hana Zoubeidi and Besma Ben Dhaou and Zohra Aydi and Aschraf Chadli Debbiche and Anissa Zaouak and Fatma Boussema},
      title = {Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge},
      journal = {International Journal of Clinical Dermatology},
      volume = {5},
      number = {2},
      pages = {11-14},
      doi = {10.11648/j.ijcd.20220502.11},
      url = {https://doi.org/10.11648/j.ijcd.20220502.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcd.20220502.11},
      abstract = {Mycosis Fongoïde is a rare T cell lymphoma. The mean age of onset is about sixty year old, mostly in masculin population. The clinical presentation is dominated by cutaneous signs. We report the case of a 78 year old patient, with a history of erythroderma with pruritus non ameliorated by corticosteroid topics, who was readmitted four years later for osteomyelopathy. At physical examination, he had bilateral axillary lymph nodes. We also noted dry generalized erythroderma with no interval of healthy skin and at the neurologic exam, there was ataxia on walking and a bilateral pyramidal syndrome majorated on the right. The blood count showed hyperleukocytosis with hyperlymphocytosis and chronic hypereosinophilia. The blood smear showed Sezary cells of the order of 2000. The CT scan has confirmed the presence of axillary adenopathies, left breast nodules not found on breast ultrasound, and bilateral adenomegaly of the external iliac and inguinal chains. The first cutaneous biopsy was not conclusive. Axillary node biopsy, 6th skin biopsy and Immunophenotyping of circulating CD4+ T lymphoctes allowed to retain Mycosis fungoides. He was treated with Methotrexate for a year with improvement. Mycosis Fungoides is a rare disease with diagnostic and therapeutic difficulties due to the non contribution of confirmatory examinations wich may delay the diagnostic and with a possibility of atypical presentation.},
     year = {2022}
    }
    

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  • TY  - JOUR
    T1  - Mycosis Fungoïdes in an Old Patient: A Diagnostic Challenge
    AU  - Roua Mankai
    AU  - Fatma Daoud
    AU  - Imene Rachdi
    AU  - Fatma Khanchel
    AU  - Mehdi Somai
    AU  - Hana Zoubeidi
    AU  - Besma Ben Dhaou
    AU  - Zohra Aydi
    AU  - Aschraf Chadli Debbiche
    AU  - Anissa Zaouak
    AU  - Fatma Boussema
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    DO  - 10.11648/j.ijcd.20220502.11
    T2  - International Journal of Clinical Dermatology
    JF  - International Journal of Clinical Dermatology
    JO  - International Journal of Clinical Dermatology
    SP  - 11
    EP  - 14
    PB  - Science Publishing Group
    SN  - 2995-1305
    UR  - https://doi.org/10.11648/j.ijcd.20220502.11
    AB  - Mycosis Fongoïde is a rare T cell lymphoma. The mean age of onset is about sixty year old, mostly in masculin population. The clinical presentation is dominated by cutaneous signs. We report the case of a 78 year old patient, with a history of erythroderma with pruritus non ameliorated by corticosteroid topics, who was readmitted four years later for osteomyelopathy. At physical examination, he had bilateral axillary lymph nodes. We also noted dry generalized erythroderma with no interval of healthy skin and at the neurologic exam, there was ataxia on walking and a bilateral pyramidal syndrome majorated on the right. The blood count showed hyperleukocytosis with hyperlymphocytosis and chronic hypereosinophilia. The blood smear showed Sezary cells of the order of 2000. The CT scan has confirmed the presence of axillary adenopathies, left breast nodules not found on breast ultrasound, and bilateral adenomegaly of the external iliac and inguinal chains. The first cutaneous biopsy was not conclusive. Axillary node biopsy, 6th skin biopsy and Immunophenotyping of circulating CD4+ T lymphoctes allowed to retain Mycosis fungoides. He was treated with Methotrexate for a year with improvement. Mycosis Fungoides is a rare disease with diagnostic and therapeutic difficulties due to the non contribution of confirmatory examinations wich may delay the diagnostic and with a possibility of atypical presentation.
    VL  - 5
    IS  - 2
    ER  - 

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Author Information
  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Department of Pathology, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Department of Pathology, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Department of Dermatology, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

  • Internal Medicine Department, Habib Thameur Hospital, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia

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