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Sézary's syndrome
Also known as:
Baccaredda-Sézary syndrome
Sézary's erythroderma
Sézary's reticulosis
Sézary-Baccaredda syndrome
Sézary-Bouvrain syndrome
Associated persons:
Aldo Baccaredda
Yves Bouvrain
Albert Sézary
Description:
A chronic skin disease which is a form of cutaneous T-cell lymphoma. It is manifested by generalized exfoliative erythroderma, intense pruritus, peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear cells in the skin, lymph nodes, and peripheral blood (Sézary cells). These cells are usually large, with cerebriform or deeply folded nucleus “cellule monstreuse” which infiltrate the skin. They are helper T lymphocytes. Facial infiltration may produce facies leonina. Some patients also have alopecia and nail dystrophy. The Sézary syndrome and mycosis fungoides are considered by some authors the same entity, and by others as two separate forms of the cutaneous T-cell lymphoma. It was first described by Sézary and Bouvrain in 1938. The condition occurs chiefly in elderly females. Very few cases reported. The description of "his" syndrome was based on 150 cases of lymphogranulomatosis he collected between 1932 and 1939.
Bibliography:
- A. Baccaredda:
Reticuloendoteliosis cutanea e malanodermia.
Atti della Societa italiana di dermatologia e sifilografia, 1937: 649-657.
- A. Sézary, Y. Bouvrain:
Erythrodérmie avec présence de cellules monstrueuses dans le derme et le sang circulant.
Bulletin de la Société francaise de dermatologie et de syphiligraphie, Paris, 1938, 45: 254-260.
- A. Baccaredda:
Reticulohistiocytosis cutanea hyperplastica beigna cum melanodermia. Beitrag zum Studium der peripheren Reticulohistiocytosen.
Archiv für Dermatologie und Syphilis, Berlin, 1939; 179: 209-256.
- G. A. Lista, R. Parcero, A. L. Aguero, R. Balabanian:
[Reticuloendotheliosis. Sezary-Baccaredda's syndrome] Article in Spanish.
La Prensa Medica Argentina, Buenos Aires, August 11, 1967, 54 (24):1174-1178.
- A. Rebora, F. Crovato, R. Bertamino, E. Nunzi:
Baccaredda-Sezary syndrome. Journal of the American Academy of Dermatology, St. Louis, November 1984, 11 (5, part 1): 907-908.
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