Cutaneous T-Cell Lymphoma (CTCL) includes a wide range of lymphoproliferative disorders that appear in the skin [1]. CTCL itself is one of the manifestations of Extra nodal Non-Hodgins Lymphoma (NHL) [2,3]. Non-Hodgins Lymphoma manifestations in other organs are known as extra nodal NHL and on the skin known as Primary Cutaneous Lymphoma. The major subtypes of CTCL includes Mycosis Fungoides (MF) and Sezary Syndrome (SS). Mycosis Fungoides is commonest among two and is described by a malignant T-cell population that is limited to the skin [1]. However, the pathogenesis of this variant is still unclear [4], it is assumed that T-cell proliferation, caused by long-term antigenic stimulation, may lead to the appearance of a malignant clone [5]. 

A 63 years old male with skin lesions on hands, back and legs was admitted to the department of dermatology in September 2018. On examination, vitals, laboratory and radiological investigations were normal. The histopathological examination of excisional biopsy revealed an infiltration of lymphocytes into the epidermal tissue. Based on peripheral blood smear (no Sezary cell was identified) and immunohistochemistry (IHC) findings (LCA, CD20, CD3, CD45, CD8 and Ki67 were positive but CD4 and CD30 were negative), the final diagnosis was CTCL and according to the modified Tumor-Node-Metastasis-Blood (TNMB) classification its type was the Mycosis Fungoides syndrome with IB stage (T2 N0 M0 B0). Thus, patient was started on interferon-α and showed a relatively good response. Patient was well for around 1 year and then defaulted for next 1 year. Patient then presented in 2020 with stage IIA disease and was referred to department of radiotherapy for further management. Here the patient was subjected to a combination chemotherapy of Cyclophosphamide, Doxorubicin, Vincristine and Prednisone (CHOP) and till now patient has received 4 cycles. At the moment, the patient has a promising condition and is undergoing our therapeutic courses.

Figure 1: Skin lesions on leg

Figure 2: Skin lesions on hands

Cutaneous T Cell Lymphoma (CTCL) is a category of varied non-Hodgkin lymphomas which demonstrates the malignancy of T cells in the skin⁷. This lymphoma includes two subtypes: Mycosis Fungoides (>65%) and Sezary syndrome [6]. The risk of MF in male: female is 2:1, the average age of the diagnosis is 55 years and incidence of this disease is about 0.4 per 100,000 person-years [7,8]. However, the main pathogenesis of this disease is still unknown, but according to recent studies, many factors can be involved in the pathogenesis of this disease, including dysfunction of some genes such as TOX or disturbance in signaling pathways such as Notch or the SMARC gene family affecting changes in histones and DNA methylation [2].

In early-stage disease, skin directed therapies are used which includes PUVA, topical bexarotene, UVB (ultraviolet B), radiotherapy, topical corticosteroids, phototherapy, nitrogen mustard (such as ifosfamide), carmustine etc. However, in advanced-stage disease Histone Deacetylase Inhibitors (HDACi), localized radiotherapy, systemic chemotherapy, monoclonal antibodies and Total Skin Electron Beam (TSEB) are the only valid treatment options available [9,10]. Interferon-α can be used in both early and advanced stages of the disease [9].

1. Alibert, J.L.M. Description des Maladies de la Peau Observées à l'Hôpital Saint-Louis, Paris. Borris, 1806, p. 157.

2. Murphy, G.F. and R. Schwarting. "Cutaneous lymphomas and leukemias." In Lever’s Histopathology of the Skin, 9th ed., Lippincott Williams and Wilkins, 2005, pp. 927–978.

3. Van Doorn, R., et al. "Mycosis Fungoides: Disease Evolution and Prognosis of 309 Dutch Patients." Archives of Dermatology, vol. 136, 2000, pp. 504-510.

4. Vergier, B., et al. "Transformation of Mycosis Fungoides: Clinicopathological and Prognostic Features of 45 Cases." Blood, vol. 95, 2000, pp. 2212–2218.

5. Kim, Y.H. and R.T. Hoppe. "Mycosis Fungoides and the Sezary Syndrome." Seminars in Oncology, vol. 26, 1999, pp. 276–289.

6. Nagatani, T., et al. "Comparative study of cutaneous T-Cell lymphoma and adult T-Cell lymphoma/leukemia: Clinical, histopathologic and immunohistochemical analysis." Cancer, vol. 66, 1990, pp. 2380–2386.

7. Barcos, M. "Mycosis Fungoides: Diagnosis and pathogenesis." Clinical Pathology, vol. 99, 1993, pp. 452–458.

8. Glusac, E.J. "Criterion by criterion: Mycosis Fungoides." American Journal of Dermatopathology, vol. 25, 2003, pp. 264–269.

9. Zackheim, H.S., et al. "Prognosis in cutaneous T-Cell lymphoma by skin stage: Long-term survival in 489 patients." Journal of the American Academy of Dermatology, vol. 40, 1999, pp. 418–425.