MELANODERMA AS AN EARLY CLINICAL MANIFESTATION OF SÉZARY SYNDROME
Keywords:
primary cutaneous lymphomas, cutaneous T-cell lymphomas, Sézary syndrome, melanodermiaAbstract
Primary cutaneous lymphomas are a heterogeneous group of hematologic malignancies originating from the T-cells or B-cells residing within the human skin. More than two-thirds (70-85%) of cases are diagnosed as cutaneous T-cell lymphomas (CTCL) with Mycosis fungoides and Sézary syndrome being the most notable. CTCL have an annual incidence of 10 cases per 1 million, with a predominance to males over the age of 50 (male-to-female ratio of 2:1).
Here, we present a case of a 54-year-old female who presented with persistent and generalized hyperpigmentation, later evolving into Sézary syndrome. Initially, the patient was reffered to the University Clinic for Dermatology in Skopje, due to a five-month medical history of persistent pruritus and hyperpigmentation. Based on the clinical presentation and skin biopsy findings consistant with non-specific dermatitis, she was diagnosed as having atopic dermatitis and underwent treatment with topical steroids and emollients. However, the clinical presentation got worsen in the following six months, and evolved into generalized melanodermia with ulcerated tumor growths. In order to conclude the final diagnosis, a biopsy of the skin lesions was performed with histopathological findings consistent with Sézary syndrome. Following this diagnosis, the patient was referred to the University Clinic for Hematology in Skopje for further treatment.
References
Miyashiro D, Sanches JA. Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management. Front Oncol. 2023 Apr 14;13:1141108. doi: 10.3389/fonc.2023.1141108.
Hristov AC, Tejasvi T, Wilcox RA. Mycosis fungoides and Sézary syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol. 2019 Sep;94(9):1027-1041. doi: 10.1002/ajh.25577.
Lee H. Mycosis fungoides and Sézary syndrome. Blood Res. 2023 Apr 30;58(S1):66-82. doi: 10.5045/br.2023.2023023.
Latzka J, Trautinger F. Mycosis fungoides and Sézary syndrome - Review and outlook. J Dtsch Dermatol Ges. 2023 Apr;21(4):386-391. doi: 10.1111/ddg.15051. Epub 2023 Mar 27.
Miyashiro D, Souza BCE, Torrealba MP, Manfrere KCG, Sato MN, Sanches JA. The Role of Tumor Microenvironment in the Pathogenesis of Sézary Syndrome. Int J Mol Sci. 2022 Jan 15;23(2):936. doi: 10.3390/ijms23020936.
García-Díaz N, Piris MÁ, Ortiz-Romero PL, Vaqué JP. Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy. Cancers. 2021;13(8):1931. doi: 10.3390/cancers13081931.
Roccuzzo G, Giordano S, Avallone G, Rubatto M, Canonico S, Funaro A, Ortolan E, Senetta R, Fava P, Fierro MT, Ribero S, Quaglino P. Sézary Syndrome: Different Erythroderma Morphological Features with Proposal for a Clinical Score System. Cells. 2022 Jan 20;11(3):333. doi: 10.3390/cells11030333.
Lu YY, Wu CH, Lu CC, Hong CH. Hyperpigmentation as a peculiar presentation of mycosis fungoides. An Bras Dermatol. 2017;92(5 Suppl 1):92-94. doi: 10.1590/abd1806-4841.20175544.
Quaglino P, Pimpinelli N, Berti E, Calzavara-Pinton P, Alfonso Lombardo G, Rupoli S, Alaibac M, Bottoni U, Carbone A, Fava P, Fimiani M, Mamusa AM, Titli S, Zinzani PL, Bernengo MG; Gruppo Italiano Linfomi Cutanei. Time course, clinical pathways, and long-term hazards risk trends of disease progression in patients with classic mycosis fungoides: a multicenter, retrospective follow-up study from the Italian Group of Cutaneous Lymphomas. Cancer. 2012;118(23):5830-9. doi: 10.1002/cncr.27627.
Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023. Eur J Cancer. 2023 Dec;195:113343. doi: 10.1016/j.ejca.2023.113343.
