Cutaneous T-cell lymphoma
3rd Nov 2021
Interview and article by Christine Clark, PhD, FRPharmS.
Cutaneous lymphomas (CLs) represent a rare and heterogeneous group of lymphomas that present in the skin without extracutaneous manifestations at the time of diagnosis.1 Mycosis fungoides (MF) and Sézary syndrome (SS) are the two best studied types of CTCL,2 together accounting for around two thirds of all CTCLs.3,4
Dr Carsten Weishaupt (Head of Dermato-oncology, University Hospital of Münster, Germany) explains that a cutaneous T-cell lymphoma (CTCL) is a rare disease with an incidence of less than one in 100,000 people per year.
MF and SS typically present differently. He says,
“We have on one hand mycosis fungoides which really starts in the skin and it usually primarily involves only the skin and then it might spread to other organs like the lymph nodes, the blood but also the visceral organs and, in contrast to that, we have the Sézary syndrome, which starts with erythroderma ….. and we also have involvement of the lymph nodes and the blood which is quite typical and it’s also more aggressive than mycosis fungoides.”
Diagnosis can be challenging. Dr Weishaupt says,
“Especially if you have erythroderma you have to think about all the different diagnoses and it often takes a long time until we really have the diagnosis of CTCL because often it is misdiagnosed with atopic dermatitis or even psoriasis sometimes”.
References
- Vermeer M. Epidemiology of cutaneous lymphoma. BJD 2021;184:1059-1067
- Orphanet. Prevalence and incidence of rare diseases: Bibliographic data. January 2020. Accessed December 2020.
- Krejsgaard T, et al. Semin Immunopathol. 2017;39:269–282.
- Trautinger F, et al. Eur J Cancer. 2017;77:57–74.