Résumé
Sezary syndrome (SS) is associated with disease and treatment-related immunosuppression. The increased incidence of cutaneous squamous cell carcinom (cSCC) in immunocompromised patients is well known, particularly in organ transplant recipients. Objective: a nationwide multicenter retrospective study was conducted to assess the incidence and characteristics of cSCC in SS patients. Material and methods: data were retrospectively collected from the files of SS patients in the dermatology departments of 19 French university hospitals, over a period of 10 to 20 years. Results: out of 582 SS included, 66 cSCC were identified during follow-up in 38 patients (overall incidence 6.5%; 1 to 12 cSCC/patient with a mean of 1.7/patient), at a mean age of 74 years for the first cSCC (33-88 years) and an average of 4 years (0-10 years) after SS diagnosis. There was a clear North/South gradient, with an incidence of 10.3% in Southern regions vs 3.2% in Northern areas. Discussion: the annual incidence of cSCC in SS appears to be significantly higher compared to general population in France (at least 0.65% (for an average 10-year follow-up) vs. 0.03%). These results are in line with a prior study (Scarisbrick et al. Arch Dermatol 1999). The reasons underlying this increased incidence are likely multifactorial: disease-related immunosuppression, treatments including chemotherapies and phototherapy and close dermatological follow-up. Conclusion: SS appears to be associated with a higher risk of cSCC compared to general population. These data warrant careful monitoring of these patients and the early implementation of photoprotective measures.