A recent study analyzed the incidence and characteristics of cutaneous squamous cell carcinoma (cSCC) in patients with Sézary syndrome.
Sézary syndrome is a rare, aggressive form of cutaneous T-cell lymphoma (CTCL), a group of rare cancers that occur when T-cells become cancerous and affect the skin. It is characterized by a widespread red rash that may cover most of the body, the presence of cancerous T cells in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin, fever, weight loss, hair loss, outward turning of the eyelids, palmoplantar keratoderma, malformation of the nails, and hepatosplenomegaly. The exact cause of Sezary syndrome is currently unknown.
cSCC is the second most common cutaneous malignancy in the United States. Risk factors include immunosuppression, chronic wounds, fair skin, male gender, older age, several genetic syndromes, environmental exposures such as UV radiation, and a previous history of squamous cell carcinoma. Although metastasis is rare, the most common site of metastasis is the lymph nodes.
Study
The objective of this retrospective survey was to assess the incidence and characteristics of cSCC in patients with Sézary syndrome. Among 582 patients analyzed, 38 received a diagnosis of at least one cSCC during follow-up. The mean interval of occurrence was four years after Sézary syndrome diagnosis, and 48% of cases occurred in the head or neck.
Based on these results, cSSC incidence seems to be higher in patients with Sézary syndrome than the general French population, where this study took place. cSSC also appears to be more aggressive in these patients and has a local recurrence rate of 13.1%. Possible factors for this increased risk are noted as disease-associated immunosuppression, the role of treatments, and UV exposure.
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References:
Guillon L, et al. Increased risk of cutaneous squamous cell carcinomas in patients with Sezary syndrome: a nationwide multicenter retrospective study. J Amer AcadDermatol. 2025; [in press], doi: https://doi.org/10.1016/ j.jaad.2025.03.049.
Hadian Y, et al. Cutaneous Squamous Cell Carcinoma. Stat Pearls [Internet], 2024. https://www.ncbi.nlm.nih.gov/books/NBK441939/
