Clonal rearrangement of the T cell receptor beta gene in the circulating lymphocytes of erythrodermic follicular mucinosis.

Follicular mucinosis is a condition characterized by the abnormal accumulation of acidic mucopolysaccharides in hair follicles. It is classically described as occurring idiopathically in young persons and within the infiltrates of mycosis fungoides in older individuals. We report a 12-year-old girl who had erythrodermic follicular mucinosis, hypereosinophilia, circulating Sezary cells, and both immunophenotypic and genotypic evidence of T cell neoplasia. Erythrodermic follicular mucinosis may represent an unusual variant of the Sezary syndrome, which to date has not been described in children or adolescents.