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CONGENITAL ICHTHYOSIS: CONCURRENT IMMUNODEEICIENCY AND ATYPICAL T CELLS |
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| Authors: | G. F. M. HENDRICKX MD B. J. M. ZEGERS Ph.D. L. VAN DELDEN M.D. J. W. STOOP M.D. |
| Affiliation: | University Children's Hospital, Het Withelmina Kinderzickenhuis, Utrecht, The Netherlands, and The Department of Subcellular Pathology, Faculty of Medicine, Free University, Amsterdam, The Netherlands |
| Abstract: | A patient with congenital ichthyosis and progressive neurologic anomalies showed disturbances in the specific humoral and cellular defense as well as the presence 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. |
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