title: The man behind the eponym dermatology in historical perspective: Albert Sézary and the Sézary syndrome.
abstract: In a series of papers from 1938 to 1949, Albert Sézary, a French dermatologist and syphilologist, described erythroderma with cellules monstrueuses (monster cells) in the skin and blood, which is now known as Sézary syndrome or Sézary disease. This historical note reprises the life and work of Sézary. It outlines his original reports and his thoughts about the pathogenesis of the disease as a reticulosis, and lists a composite classification of the reticuloses, which includes that of Sézary. We touch briefly on the articles that first used the terms Sézary reticulosis and Sézary syndrome and the changing concepts of the reticuloses and the reticuloendothelial system. We conclude that Sézary syndrome (Sézary disease, Sézary reticulosis) cannot be separated from mycosis fungoides clinically, histopathologically, hematologically, or viscerally and, therefore, is not a disease sui generis. Despite our conclusions, present day consensus defines Sézary disease clinically as a generalized pruritic erythroderma: histopathologically with an epidermal and dermal infiltrate, lymphadenopathy and visceral involvement all containing monster cells (Lutzner/Sézary cells) in the skin, peripheral blood, lymph nodes, and viscera, a disease different and separate from mycosis fungoides.
authors.forename: Charles
authors.initials: C
authors.lastname: Steffen
mesh_terms: D003880:Dermatology / Q000266:history*; D004850:Eponyms; D005602:France; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D012751:Sezary Syndrome / Q000266:history* / Q000473:pathology*