Abstract
Turner syndrome (TS) is the most common sex chromosome abnormality among females, characterized by short stature, hypergonadotropic hypogonadism, congenital heart anomalies, and an increased risk of autoimmune diseases. Although TS does not typically increase the absolute risk of malignancy, specific cancers, such as those affecting the nervous system and gastrointestinal tract and malignant melanoma, may occur more frequently. Mycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphoma, generally affecting otherwise healthy adults but also seen in children and adolescents. We report an 11.2-year-old girl with TS presenting with substantial weight gain and short stature. Clinical examination revealed characteristic TS features and karyotype analysis confirmed mosaic TS. Following growth hormone (GH) therapy, the patient developed persistent, erythematous, itchy skin lesions diagnosed as folliculotropic MF. GH therapy was discontinued, and topical steroids controlled the skin lesions effectively. MF in TS is very rare and unexpected, especially in a child. This is the first reported case of MF in a child with TS. This case highlights the importance of carefully evaluating skin lesions in patients with TS and suggests considering MF as a differential diagnosis.
Keywords: Turner syndrome, mycosis fungoides, malignancy, primary cutaneous T-cell lymphoma
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References
Gravholt CH, Viuff MH, Brun S, Stochholm K, Andersen NH. Turner syndrome: mechanisms and management. Nat Rev Endocrinol. 2019;15:601-614. Epub 2019 Jun 18
Kim SE, Park SH, Han K, Cho WK, Suh BK, Park YG. Population prevalence, cancer risk, and mortality risk of turner syndrome in South Korean women based on National Health Insurance service data. Yonsei Med J. 2022;63:991-998.
Viuff MH, Stochholm K, Lin A, Berglund A, Juul S, Gravholt CH. Cancer occurrence in Turner syndrome and the effect of sex hormone substitution therapy. Eur J Endocrinol. 2021;184:79-88.
Baykal C, Atcı T, Pehlivan Ulutaş G. Primary cutaneous lymphomas in pediatric age group: experience of a single dermatology center. Turk Arch Pediatr. 2023;58:229-231.
Cervini AB, Torres-Huamani AN, Sanchez-La-Rosa C, Galluzzo L, Solernou V, Digiorge J, Rubio P. Mycosis fungoides: experience in a pediatric hospital. Actas Dermosifiliogr. 2017;108:564-570. Epub 2017 Mar 7
Cerroni L. Mycosis fungoides-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018;37:2-10.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768-3785. Epub 2005 Feb 3
Yazganoglu KD, Topkarci Z, Buyukbabani N, Baykal C. Childhood mycosis fungoides: a report of 20 cases from Turkey. J Eur Acad Dermatol Venereol. 2013;27:295-300. Epub 2011 Dec 19
Willemze R. Mycosis fungoides variants-clinicopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018;37:11-17.
Hodak E, Amitay-Laish I. Mycosis fungoides: a great imitator. Clin Dermatol. 2019;37:255-267. Epub 2019 Jan 11
Joshi TP, Black TA, Fernandez B, Friske S, Stafford H, Strouphauer E, Duvic M. Comorbidities associated with mycosis fungoides: a case-control study in the All of Us database. J Am Acad Dermatol. 2023;88:686-688. Epub 2022 Jul 8
Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA; UK Clinical Cytogenetics Group. Cancer incidence in women with Turner syndrome in Great Britain: a national cohort study. Lancet Oncol. 2008;9:239-246. Epub 2008 Feb 20
Ji J, Zöller B, Sundquist J, Sundquist K. Risk of solid tumors and hematological malignancy in persons with Turner and Klinefelter syndromes: a national cohort study. Int J Cancer. 2016;139:754-758. Epub 2016 Apr 19
Gravholt CH, Andersen NH, Christin-Maitre S, Davis SM, Duijnhouwer A, Gawlik A, Maciel-Guerra AT, Gutmark-Little I, Fleischer K, Hong D, Klein KO, Prakash SK, Shankar RK, Sandberg DE, Sas TCJ, Skakkebæk A, Stochholm K, van der Velden JA; International Turner Syndrome Consensus Group; Backeljauw PF. Clinical practice guidelines for the care of girls and women with Turner syndrome. Eur J Endocrinol. 2024;190:G53-G151.


