Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab.
Kimura disease (KD) is a rare condition that predominantly affects young middle-aged Asian men. It is classically characterized by tumors in the head and neck region with associated eosinophilia and elevated serum immunoglobulin E levels. The exact pathogenesis of this condition remains unknown. Although some regard it as a reactive condition, others believe that it is a T-cell-mediated disease. T-cell clonality has been recently demonstrated in a few cases. We report a 37-year-old Lebanese woman who had both KD and mycosis fungoides (MF). T-cell receptor gene rearrangement studies using 2 different techniques did not detect any clonality for T-cell receptor in both KD and MF lesions. Due to the presence on histology of lymphoid follicles and the persistent high serum immunoglobulin E levels, we elected to attempt treatment with rituximab. Although the KD lesions persisted, they became softer and less nodular. As for the MF lesions, they flared transiently and then exhibited a sustained improvement over a follow-up period of 1 year.
Ghosn
Samer
S
Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon. sg03@aub.edu.lb
Bahhady
Ruba
R
Mahfouz
Rami
R
Abbas
Ossama
O
Kibbi
Abdul Ghani
AG
Saad
Rana
R
Taher
Ali
A
D000328:Adult; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D005260:Female; D005803:Genes, Immunoglobulin; D019672:Genes, T-Cell Receptor; D006801:Humans; D007861:Lebanon; D009182:Mycosis Fungoides / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D000069283:Rituximab; D012878:Skin Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab