Sezary syndrome and Leser-Trelat sign: A chance association?
Fiche publication
Date publication
avril 2008
Auteurs
Membres identifiés du Cancéropôle Est :
Dr GRANEL-BROCARD Florence
Tous les auteurs :
Bursztejn AC, Doumat-Batch F, Granel-Brocard F, Perrin A, Cuny JF, Barbaud A, Schmutz JL
Lien Pubmed
Résumé
Background. - Leser-Trelat sign involves the combined sudden onset of seborrheic keratosis and cancer. However, some doubt surrounds the existence of this syndrome. We report a case of Leser-Trelat sign that led to the discovery of Sezary syndrome. Patients and methods. - A 59-year-old woman presented generalized pruritus with secondary appearance of multiple seborrheic keratosis. Leser-Trelat sign was diagnosed and 20 months later, Sezary syndrome was discovered. Extracorporeal photopheresis was initiated, after which there was a marked reduction in the patient's pruritus, erythroderma and numbers of seborrheic keratoses and Sezary. cells. Discussion. - Leser-Trelat sign is often associated with gastric carcinoma or lymphoproliferative tumours. Rampen and Schwengle [J Am Acad Dermatol 21 (1989) 50-5] have thrown doubt on this entity because of the "subjective" definition, the frequent dissociation between the course of the tumour and that of the seborrheic keratosis, the disparity between the frequency with which rapid onset seborrheic keratosis is seen and the rarity of cases in which this phenomenon reveals a tumour and the absence of association with any specific type of malignancy. The time between diagnosis of Sezary syndrome and cutaneous symptoms of Leser-Trelat sign appears very tong in the present case. In the absence of any established physiopathology, it is impossible to prove any direct link between these two syndromes. Leser-Trelat sign remains controversial. Knowledge of its pathogenesis could help determine whether Leser-Trelat sign should or should not be considered a paraneoplastic syndrome. (C) 2008 Elsevier Masson SAS. Tous droits reserves.
Référence
Ann Dermatol Venereol. 2008 Apr;135(4):287-90