The clinical course of a patient with SÚzary syndrome presenting under bullous form is described. The bullae were flaccid on a non-erythematous base. Indirect and direct immunofluorescence were both negative. In the peripheral blood WBC was high (54,000/mm3) and large cells with T lymphocyte properties and small cells with 'null' cell features were present. Blister fluid contained only large, E-rosette positive and alpha-naphthylacetate esterase-positive SÚzary cells. A remarkable spontaneous cyclicity was observed: marked reduction in the number of large circulating SÚzary cells each time bullae appeared. This suggests that only the large cells were capable of migrating to the skin and from the skin to peripheral blood.
Bullous Sézary syndrome.
BERNENGO, Maria Grazia;
1981-01-01
Abstract
The clinical course of a patient with SÚzary syndrome presenting under bullous form is described. The bullae were flaccid on a non-erythematous base. Indirect and direct immunofluorescence were both negative. In the peripheral blood WBC was high (54,000/mm3) and large cells with T lymphocyte properties and small cells with 'null' cell features were present. Blister fluid contained only large, E-rosette positive and alpha-naphthylacetate esterase-positive SÚzary cells. A remarkable spontaneous cyclicity was observed: marked reduction in the number of large circulating SÚzary cells each time bullae appeared. This suggests that only the large cells were capable of migrating to the skin and from the skin to peripheral blood.
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