Citometría de Flujo y PCR por rearreglo del TCR en sangre, son importantes para la detección del Síndorme de Sézary en forma temprana.

Nonerythrodermic Dermatitis and Early Sézary Syndrome

J Am Acad Dermatol; ePub 2017 Jul 11; Mangold, et al

July 19, 2017

Erythroderma is uncommon as an initial sign of Sézary syndrome (SS), according to a recent study. Therefore, 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. A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015. Researchers found:

• 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.

Citation:

Mangold AR, Thompson AK, Davis MD, et al. Early clinical manifestations of Sézary syndrome: A multicenter retrospective cohort study. [Published online ahead of print July 11, 2017]. J Am Acad Dermatol. doi:10.1016/j.jaad.2017.05.036.