Peer Reviewed

Photoclinic

Mycosis Fungoides

MUHAMMAD WASIF SAIF, MD

Volume 56 - Issue 11 - November 2016

AUTHORS:
Kaitlin Dougherty and Shailendra Saxena, MD, PhD
Creighton University School of Medicine, Omaha, Nebraska

CITATION:
Dougherty K, Saxena S. Mycosis fungoides. Consultant. 2016;56(11):1037.

 

Two years ago, a 55-year-old woman presented with a persistent rash measuring approximately 10 × 17 cm on her right lower back. The rash was erythematous and flaking, with the central portion progressing to a purple color. At initial presentation, the patient had been given a topical corticosteroid cream to which the rash had responded well.

Two years later, the patient presented again with the same rash. This time, the rash had spread from her right lower back (Figure) to other parts of the body, including her right arm and upper back. She was sent to a dermatologist who ordered a biopsy, the results of which confirmed a diagnosis of mycosis fungoides (MF).

The patient was prescribed topical clobetasol, 0.05%, applied twice daily, and oral dexamethasone, to be taken for 21 days with a tapering dosage. At a follow-up visit after 4 weeks, there had been no significant improvement in the rash with the medication.

The diagnosis of MF can be especially concerning to patients, because its name is a misnomer that can lead patients to believe that they have a fungal infection. In actuality, MF is the most common type of cutaneous T-cell lymphoma.1 This news can be even more alarming to patients, because they know that lymphoma is cancer.

It is very important to educate patients about the condition and help them understand that MF is a malignant cancer, but that it does not progress very often. A recent analysis of studies enrolling nearly 6000 patients with MF found that the mean overall 10-year survival rate (taking into account all stages of the cancer) was approximately 70%. Stage T1 MF (covering <10% of body surface) had a mean 10-year survival of approximately 86%.2 Stage T2 MF (covering >10% of body surface) was found to have a mean 10-year survival of 67%. Stage T3 (1 or more tumors ≥1 cm in diameter) and stage T4 (covering ≥80% of body surface) disease had worse prognoses, with mean 10-year survival rates of 34% and 27%, respectively.2 Our patient’s MF can be classified as stage T1, since the rash covered less than 10% of her body. With these survival rates in mind, it is best not to scare patients, but they should be made aware of the potential progression of the disease and watch it closely.

References:

  1. Desai M, Liu S, Parker S. Clinical characteristics, prognostic factors, and survival of 393 patients with mycosis fungoides and Sézary syndrome in the southeastern United States: a single-institution cohort. J Am Acad Dermatol. 2015;72(2):​276-285.
  2. Vollmer RT. A review of survival in mycosis fungoides. Am J Clin Pathol. 2014;​141(5):706-711.
References

1. Siegel RS, Pandolfino T, Guitart J, et al. Primary cutaneous T-cell lymphoma: review and current concepts. J Clin Oncol. 2000;18:2908-2925.
2. Poiesz BJ, Ruscetti FW, Gazdar AF, et al. Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma. Proc Natl Acad Sci U S A. 1980;77:7415-7419.
3. Gelliarth KA, DeSantis P. A brief review of the pathophysiology and treatment of cutaneous T-cell lymphoma "mycosis fungoides." Ostomy Wound Manage. 1995;41:44-48.
4. Duncan KO, Heald PW. T-cell technology in the diagnosis & management of cutaneous T-cell lymphoma. Compr Ther. 1998;24:117-122.
5. Weiss LM, Hu E, Wood GS, et al. Clonal rearrangements of T-cell receptor genes in mycosis fungoides and dermatopathic lymphadenopathy. N Engl J Med. 1985;313:539-544.
6. Bakels V, van Oostveen JW, Gordijn RL, et al. Di-agnostic value of T-cell receptor beta gene rearrangement analysis on peripheral blood lymphocytes of patients with erythroderma. J Invest Dermatol. 1991; 97:782-786.
7.Guitart J, Kaul K. A new polymerase chain reaction-based method for the detection of T-cell clonality in patients with possible cutaneous T-cell lymphoma. Arch Dermatol. 1999;135:158-162.
8. Russell-Jones R, Whittaker S. T-cell receptor gene analysis in the diagnosis of Sézary syndrome. Acad Dermatol. 1999;41:254-259.
9. Sausville EA, Eddy JL, Makuch RW, et al. Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups. Ann Intern Med. 1998;109:372-382.
10. Dmitrovsky E, Matthews MJ, Bunn PA, et al. Cytologic transformation in cutaneous T cell lymphoma: a clinicopathologic entity associated with poor prognosis. J Clin Oncol. 1987;5:208-215.