Sézary syndrome presenting as a follicular dermatosis

A patient with erythroderma initially characterized by follicular papular dermatosis was diagnosed clinically and histologically as having probable pityriasis rubra pilaris. In addition the patient had an elevated WBC with absolute lymphocytosis. Ninety percent of the lymphocytes had the features of Sézary cells by light and electron microscopy. He was diagnosed as having Sézary's syndrome despite the noncharacteristic findings in the skin biopsy. This case illustrates that Sézary's syndrome can present initially as a follicular dermatosis.