Aaron R Mangold1, Agnieszka K Thompson2, Mark D Davis3, Ieva Saulite4, Antonio Cozzio5, Emmanuella Guenova5, Emmilia Hodak6, Iris Amitay-Laish6, Ramon M Pujol7, Mark R Pittelkow8, Robert Gniadecki9. 1. Mayo Clinic, Scottsdale, Arizona. Electronic address: mangold.aaron@mayo.edu. 2. Mayo Clinic, Rochester, Minnesota; New York University, New York, New York. 3. Mayo Clinic, Rochester, Minnesota. 4. Department of Dermatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. 5. Department of Dermatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland; Kantonspital St. Gallen, St. Gallen, Switzerland. 6. Department of Dermatology, Rabin Medical Center, University of Tel-Aviv, Petah Tikva, Israel. 7. Department of Dermatology, Hospital del Mar, Universitat Autònoma de Barcelona, Barcelona, Spain. 8. Mayo Clinic, Scottsdale, Arizona. 9. University of Alberta, Edmonton, Canada; Department of Dermatology, University of Copenhagen, Copenhagen, Denmark.
Abstract
BACKGROUND: Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease. OBJECTIVE: To describe the early clinical characteristics of patients with SS. METHODS: A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015. RESULTS: Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation. LIMITATIONS: This study is retrospective. CONCLUSION: Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.
BACKGROUND: Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease. OBJECTIVE: To describe the early clinical characteristics of patients with SS. METHODS: A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015. RESULTS:Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation. LIMITATIONS: This study is retrospective. CONCLUSION:Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.
Authors: Maria Estela Martinez-Escala; Alba L Posligua; Heather Wickless; Audrey Rutherford; Kimberly A Sable; Belen Rubio-Gonzalez; Xiaolong A Zhou; Jason B Kaplan; Barbara Pro; Jaehyuk Choi; Christiane Querfeld; Steven T Rosen; Joan Guitart Journal: J Am Acad Dermatol Date: 2018-01-04 Impact factor: 11.527
Authors: Elena Netchiporouk; Jennifer Gantchev; Matthew Tsang; Philippe Thibault; Andrew K Watters; John-Douglas Matthew Hughes; Feras M Ghazawi; Anders Woetmann; Niels Ødum; Denis Sasseville; Ivan V Litvinov Journal: Oncotarget Date: 2017-10-07