Mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) is hard to spot in its early stages.
To get patients the help they need, it's important to diagnose as early as possible.
Importance of Multiple Biopsies3
Performing multiple biopsies is key to identifying MF-CTCL
SEE WHY
Challenges in diagnosing MF-CTCL
MF-CTCL may be misdiagnosed because of disease similarity and variability
Similarity4,5
MF-CTCL may mimic:
  • Dermatitis
  • Psoriasis
  • Eczema
  • Tinea infections
  • Eczematous/systemic drug eruptions
  • Small plaque parapsoriasis
Disease Variability
  • Various clinical manifestations of MF-CTCL may be present by themselves or occur simultaneously6
  • Due to the heterogeneous nature of the disease, multiple biopsies in different anatomical sites may
    be necessary to identify MF-CTCL3

Signs and symptoms suggestive of MF-CTCL
When diagnosing MF-CTCL, look for patches, plaques, tumors, and erythroderma7
Patch
Patches
Patches, a sign of early-stage MF-CTCL (Stage IA, IB, and IIA), are lesions of any size without significant elevation or induration. They may show pigmentation changes, scaling, crusting, and/or poikiloderma.8
Plaque
Plaques
Plaques, a sign of early-stage MF-CTCL (Stage IA, IB, and IIA), are lesions of any size that are elevated or indurated. They may show pigmentation changes, scaling, crusting, or follicular prominence; ulceration is important to document.8
Tumor
Tumors
Tumors are solid or nodular lesions ≥1 cm in diameter with evidence of depth and/or vertical growth.8 They may be ulcerated.7
Erythroderma
Erythroderma
A symptom of Sézary Syndrome, a leukemic variant of CTCL, erythroderma manifests as a confluence of erythematous lesions covering ≥80% of the body.8 It may or may not be associated with involvement of the blood.9
For more comprehensive information, please see the NCCN Guidelines.8
Image courtesy of DermNet NZ. Used with permission.

Differential diagnosis
Take on the challenge — identify which of these inflammatory skin diseases is early-stage MF-CTCL
Select an image to reveal the answer
Example of Tinea Corporis10
Example of Psoriasis11
CORRECT!
Differential diagnosis of MF-CTCL: Clinical features consistent with MF-CTCL include multiple erythematous,
well-demarcated lesions varying in shape and size (≥5 cm in diameter).12
Example of Eczema13

Raising your clinical index of suspicion
For early and accurate diagnosis of MF-CTCL, consider history, location, morphology, and pathology*12
History
The persistent nature of MF-CTCL may be an important aspect of the patient’s medical history12
Location
Predominant locations of MF-CTCL versus other inflammatory skin conditions14,15
Atopic Dermatitis
Common Locations of Atopic Dermatitis
Psoriasis
Common Locations of Psoriasis
Mycosis Fungoides
Common Locations of Mycosis Fungoides
MF-CTCL tends to be seen on sun-protected areas of the body (other areas of the skin may also be affected)4
Mycosis Fungoides
Morphology1,7,12
Morphologic Patch
Patch
  • Scaly, erythematous, atrophic patch
  • Individual lesions may vary in size, shape, and pigment, but are typically ≥5 cm
Morphologic Plaque
Plaque
  • Scaly, infiltrative, coalescing plaques, with some associated erythema
  • More generalized distribution
Morphologic Tumor
Tumor
  • Smooth-surfaced tumor with focal areas of superficial ulceration
Pathology*1,12
Pathologic Patch
Patch
  • Mild band-like infiltrate in superficial dermis with papillary dermis fibrosis and atypical haloed lymphocytes scattered along dermoepidermal junction
Pathologic Plaque
Plaque
  • Dense, thick, band-like infiltrate in middle and upper dermis
  • Marked atypical lymphocytes are present with irregular nuclear contours and perinuclear halos
Pathologic Tumor
Tumor
  • Dermal infiltrate composed of lymphocytes with hyperchromatic and pleomorphic nuclei, and prominent nucleoli
  • Typical and atypical
    mitotic figures
*See NCCN Guidelines for a more comprehensive reference for full workups.8
Images courtesy of Peter W. Heald, MD, Amy C. Musiek, MD, and Theresa R. Pacheco, MD.

Importance of multiple biopsies
“Multiple biopsies may be necessary to capture the pathologic variability of disease at diagnosis.”
— Peter W. Heald, MD16
  • When evaluating which type of biopsy may be appropriate, consider the type of lesion3
    • A shave biopsy samples a broader area of the epidermis and is typically used for thin lesions17
    • For thicker lesions, a punch biopsy may be more fitting17
  • Biopsies are more accurate when topical treatments have been discontinued 2-4 weeks prior to performing the biopsy12
A single negative biopsy may not be
sufficient to reach a conclusive diagnosis —
biopsies in different locations
may be necessary3
Shave Biopsy
Punch Biopsy

It's important to diagnose MF-CTCL as early as
possible because the disease can be a significant
challenge for patients18,19
MF-CTCL is a serious disease
  • Even in early-stage disease, MF-CTCL is associated with a significant reduction in health-related quality of life20
  • The course of early-stage MF-CTCL can be variable and unpredictable, particularly if plaques are present18
MF-CTCL can cause severe physical suffering20
  • Pruritus is the most frequent and earliest symptom of MF-CTCL21
    • Pruritus may not be relieved by emollients or other treatments22
    • In advanced stages of the disease, patients commonly report severe, diffuse pruritus that they describe as “burning pain”23
  • Patients also tend to suffer hair loss at lesion sites7
MF-CTCL can cause significant psychosocial impact
  • In earlier stages, patients endure the appearance of patches or plaques on their skin, which may be difficult to explain to family and friends20

Treatment Considerations
Therapy selection is multifactorial24
  • Probability of achieving desired goals
  • Availability of therapies
  • Comorbidities
  • Location of disease
  • Ability to tolerate adverse events
  • Experience of the treating physician
Treatment options8
  • Topical corticosteroids
  • Topical chemotherapy
  • Retinoids
  • Phototherapy (PUVA or UVB therapy)
  • Radiation therapy
  • Systemic therapies
No universally accepted treatment standard exists.25
LEARN MORE AT CLFOUNDATION.ORG
SEE NCCN GUIDELINES FOR MORE
COMPREHENSIVE INFORMATION8
REFERENCES
1. Ahn CS, ALSayyah A, Sangüeza OP. Mycosis fungoides: An updated review of clinicopathologic variants. Am J Dermatopathol. 2014;36(12):933-951. 2. van Doorn R, van Haselan CW, van Voorst Vader P, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136:504-510. 3. Elston DM, Stratman EJ, Miller SJ. Biopsy issues in specific diseases. J Am Acad Dermatol. 2016;74:1-16. 4. Mark LA. Mycosis fungoides: Diagnosis and work-up of early stage disease. Practical Dermatology website. http://practicaldermatology.com/2010/03/mycosis-fungoides-diagnosis-and-work-up-of-earlystage-disease. Published March 2010. Accessed March 7, 2017. 5. Nashan D, Faulhaber D, Ständer S, Luger TA, Stadler R. Mycosis fungoides: a dermatological masquerader. Br J Dermatol. 2007;156:1-10. 6. Cutaneous Lymphoma Foundation. CTCL-MF Fast Facts. http://www.clfoundation.org/sites/default/files/content/CL_fast_facts.pdf. Accessed March 7, 2017. 7. Hoppe et al. 2015. Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides. In: UpToDate, Rosmarin A (Ed), UpToDate, Waltham, MA. 8. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) T-cell Lymphomas. V2.2017. ©2017 NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, NCCN GUIDELINES®, and all other NCCN content are trademarks owned by the National Comprehensive Cancer Network, Inc. 9. Olsen E, Vonderheid E, Pimpinelli N. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713-1722. 10. Science Source. Tinea corporis: ringworm lesion on skin. http://images.sciencesource.com/preview/10894690/SD0166.html. Accessed May 10, 2017. 11. DermIS. Psoriasis Vulgaris, Chronic Stationary Type. http://www.dermis.net/dermisroot/en/1197237/image.htm. Accessed May 10, 2017. 12. Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. 2005;53:1053-1063. 13. Science Source. Eczema. http://images.sciencesource.com/preview/10142512/SA2631.html. Accessed May 10, 2017. 14. Weidinger S, Novak N. Atopic dermatitis. Lancet. 2016;387(10023):1109-1122. 15. Psoriasis: more than skin deep. Harv Men's Health Watch. 2010 Jul;14(12):4-5. 16. "Interview with Dr. Peter Heald." Telephone Interview. 13 May 2016. 17. Pickett H. Shave and punch biopsy for skin lesions. Am Fam Physician. 2011;84(9):995-1002. 18. Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: Validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol. 2010;28:4730-4739. 19. Song S, Willemze R, Swerdlow S. Report of the 2011 Society for Hematopathology/European Association for Haematopathology Workshop. Am J Clin Pathol. 2013;139:466-490. 20. Demierre M-F, Gan S, Jones J, Miller DR. Significant impact of cutaneous T-cell lymphoma on patients’ quality of life. Cancer. 2006;107:2504-2511. 21. Pujol R, Gallardo F, Llistosella E. Invisible mycosis fungoides: a diagnostic challenge. J Am Acad Dermatol. 2002;47(2 Suppl):S168-S171. 22. Meyer N, Paul C, Misery L. Pruritus in cutaneous T-cell lymphomas: frequent, often severe and difficult to treat. Acta Derm Venereol. 2010;90:12-17. 23. Misery L. Chapter 8: Pruritus in cutaneous T-cell lymphomas. In: Carstens E, Akiyama T, eds. Itch: Mechanisms and Treatment. Boca Raton, FL: CRC Press; 2014. 24. Poligone B, Heald P. Menus for managing patients with cutaneous T-cell lymphoma. Semi Cutan Med Surg. 2012;31(1):25-32. 25. Trautinger F, Eder J, Assaf C. European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome — Update 2017. Eur J Cancer. 2017;77:57-74.

MF-CTCL FAQ

What type of cancer is MF-CTCL?

How many cases of MF-CTCL exist in the United States?

Who has a high risk of developing MF-CTCL?

Could incidence of MF-CTCL be underreported?

REFERENCES
1. Lymphoma Research Foundation. Getting the facts: Cutaneous T-cell Lymphoma (CTCL). http://www.lymphoma.org/atf/cf/%7Baaf3b4e5-2c43-404c-afe5-fd903c87b254%7D/LRF_FACTSHEET_CTCL_2013.PDF. Accessed March 7, 2017. 2. Cutaneous Lymphoma Foundation. A Patient’s Guide to Understanding Cutaneous Lymphoma. https://www.clfoundation.org/sites/default/files/Guide%20Final_0.pdf. Accessed March 15, 2017. 3. Lymphoma Research Foundation. Cutaneous T-cell Lymphoma (CTCL). http://www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300151. Accessed March 15, 2017. 4. Wilson LD, Hinds GA, Yu JB. Age, race, gender, stage and the incidence of cutaneous lymphoma. Clin Lymphoma Myeloma Leuk. 2012;12(5):291-296.
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