CONGENITAL ICHTHYOSIS: CONCURRENT IMMUNODEEICIENCY AND ATYPICAL T CELLS

Abstract
A patient with Congenital ichthyosis and progressive anomalies showed disturbances in the specific humoral and cellular defense as well as the presemce of atypical lymphoid cells in skin and lymph node. The latter resembled the atypical T cells found in mycosis fungoides and Sézary syndrome. The possibility of the presence of either a cutaneous T cell lymphoma or unregulated T cell stimulation leading to concurrent immunodeficiency in this patient is discussed.