Journal of General Internal Medicine

, Volume 34, Issue 11, pp 2669–2674 | Cite as

An Elusive Case of Mycosis Fungoides: Case Report and Review of the Literature

  • Vincent A. PallazolaEmail author
  • Gerard Deib
  • Soni Abha
  • Rabih M. Geha
  • Kimiyoshi Kobayashi
Clinical Practice: Clinical Vignettes


Erythroderma refers to a spectrum of skin diseases resulting in diffuse erythema and scaling encompassing ≥ 90% of the body surface area. The differential diagnosis ranges from primary dermatologic diseases such as atopic dermatitis and psoriasis to potentially deadly causes such as staphylococcal toxic shock syndrome, toxic epidermal necrolysis, and malignancy. Cutaneous T cell lymphoma (CTCL) is an uncommon but highly morbid cause of erythroderma. This non-Hodgkin lymphoma remains a diagnostic challenge due to its variable clinical presentation and varied histologic features. Mycosis fungoides (MF) is the most common form of CTCL. Making a timely diagnosis is challenging as it may mimic inflammatory diseases of the skin including eczema, psoriasis, lichen planus, and cutaneous lupus. We present a case of a 58-year-old man who presented with 5 years of cutaneous symptoms and several months of fevers and night sweats, ultimately diagnosed as MF. Owing to diffuse CD30 positivity, he was a candidate for brentuximab vedotin, an antibody-drug conjugate medication that selectively targets the CD30 antigen. This resulted in an excellent therapeutic response.


cancer dermatology clinical image 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they do not have a conflict of interest.


  1. 1.
    Sigurdsson V, Steegmans PH, van Vloten WA. The incidence of erythroderma: a survey among all dermatologists in The Netherlands. J Am Acad Dermatol. 2001;45(5):675–678. doi: CrossRefPubMedGoogle Scholar
  2. 2.
    Schwartz RA, Leevy CM, Cohen PJ, Lambert WC. Erythroderma with fulminant hepatitis: a possible association. Cutis. 1986;37(1):56–58.PubMedGoogle Scholar
  3. 3.
    Rym BM, Mourad M, Bechir Z, et al. Erythroderma in adults: a report of 80 cases. Int J Dermatol. 2005;44(9):731–735. doi: CrossRefPubMedGoogle Scholar
  4. 4.
    Yamashita M, Fujii Y, Ozaki K, et al. Human immunodeficiency virus-positive secondary syphilis mimicking cutaneous T-cell lymphoma. Diagn Pathol. 2015;10:185. doi: CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Ortigosa YM, Bendazzoli PS, Barbosa AM, Ortigosa LCM. Early malignant syphilis. An Bras Dermatol. 2016;91(5 Suppl 1):148–150. doi: CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Haneke E. Nail psoriasis: clinical features, pathogenesis, differential diagnoses, and management. Psoriasis (Auckl). 2017;7:51–63. doi: CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Patel S, Zirwas M, English JC. Acquired palmoplantar keratoderma. Am J Clin Dermatol. 2007;8(1):1–11.CrossRefGoogle Scholar
  8. 8.
    Vonderheid EC. On the diagnosis of erythrodermic cutaneous T-cell lymphoma. J Cutan Pathol. 2006;33 Suppl 1:27–42. doi: CrossRefPubMedGoogle Scholar
  9. 9.
    Dalal MR, Mitchell SA, McCloskey C, Zagadailov EA, Gautam A. Epidemiological and humanistic burden of cutaneous T-cell lymphomas: results of a systematic review. Hematological Oncology. 2017;35(S2):389–390. doi: CrossRefGoogle Scholar
  10. 10.
    Sakamoto FH, Colleoni GWB, Teixeira SP, et al. Cutaneous T-cell lymphoma with HTLV-I infection: clinical overlap with adult T-cell leukemia/lymphoma. International Journal of Dermatology. 2006;45(4):447–449. doi: CrossRefPubMedGoogle Scholar
  11. 11.
    Pancake BA, Zucker-Franklin D, Coutavas EE. The cutaneous T cell lymphoma, mycosis fungoides, is a human T cell lymphotropic virus-associated disease. A study of 50 patients. J Clin Invest. 1995;95(2):547–554.CrossRefGoogle Scholar
  12. 12.
    van Doorn R, Van Haselen CW, van Voorst Vader PC, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136(4):504–510.PubMedGoogle Scholar
  13. 13.
    Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. 2005;53(6):1053–1063. doi: CrossRefPubMedGoogle Scholar
  14. 14.
    Willemze R, Hodak E, Zinzani PL, Specht L, Ladetto M, ESMO Guidelines Working Group. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2013;24 Suppl 6:vi149–154. doi: CrossRefPubMedGoogle Scholar
  15. 15.
    Krejsgaard T, Odum N, Geisler C, Wasik MA, Woetmann A. Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome. Leukemia. 2012;26(3):424–432. doi: CrossRefPubMedGoogle Scholar
  16. 16.
    Vonderheid EC, van Voorst Vader PC. Herpes zoster-varicella in cutaneous T-cell lymphomas. Arch Dermatol. 1980;116(4):408–412.CrossRefGoogle Scholar
  17. 17.
    Axelrod PI, Lorber B, Vonderheid EC. Infections complicating mycosis fungoides and Sézary syndrome. JAMA. 1992;267(10):1354–1358.CrossRefGoogle Scholar
  18. 18.
    Arulogun SO, Prince HM, Ng J, et al. Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation. Blood. 2008;112(8):3082–3087. doi: CrossRefPubMedGoogle Scholar
  19. 19.
    Benner MF, Jansen PM, Vermeer MH, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood. 2012;119(7):1643–1649. doi: CrossRefPubMedGoogle Scholar
  20. 20.
    Prince HM, Kim YH, Horwitz SM, et al. Brentuximab vedotin or physician’s choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): an international, open-label, randomised, phase 3, multicentre trial. Lancet. 2017;390(10094):555–566. doi: CrossRefPubMedGoogle Scholar

Copyright information

© Society of General Internal Medicine 2019

Authors and Affiliations

  • Vincent A. Pallazola
    • 1
    Email author
  • Gerard Deib
    • 2
  • Soni Abha
    • 3
  • Rabih M. Geha
    • 4
    • 5
  • Kimiyoshi Kobayashi
    • 1
  1. 1.Department of MedicineJohns Hopkins University School of MedicineBaltimoreUSA
  2. 2.Department of RadiologyJohns Hopkins University School of MedicineBaltimoreUSA
  3. 3.Department of DermatologyJohns Hopkins University School of MedicineBaltimoreUSA
  4. 4.Department of MedicineUniversity of California San FranciscoSan FranciscoUSA
  5. 5.Medical Service, San Francisco Veterans Affairs Medical CenterSan FranciscoUSA

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