title: Sézary syndrome without erythroderma.
abstract: BACKGROUND
  Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement.
  
  
  OBJECTIVE
  We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma.
  
  
  METHODS
  Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied.
  
  
  RESULTS
  Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred.
  
  
  CONCLUSIONS
  Retrospective design and small sample size are limitations.
  
  
  CONCLUSIONS
  Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.
authors.affiliation: Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Dermatology Department, Paris, France.
authors.forename: Aurélia
authors.initials: A
authors.lastname: Henn
authors.affiliation: Institut National de la Santé et de la Recherche Médicale (INSERM) U976, Hôpital Saint-Louis, Paris, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Michel
authors.affiliation: Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Dermatology Department, Paris, France; Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Fite
authors.affiliation: Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France; Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Pathology Department, Paris, France.
authors.forename: Lydia
authors.initials: L
authors.lastname: Deschamps
authors.affiliation: APHP, Groupe Hospitalier Henri-Mondor, Pathology Department, Créteil, France; Université Paris Est-Créteil, Créteil, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ortonne
authors.affiliation: APHP, Groupe Hospitalier Henri-Mondor, Dermatology Department, Créteil, France.
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France; Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Pathology Department, Paris, France.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Marinho
authors.affiliation: Centre Hospitalo-Universitaire (CHU) de Bordeaux, Dermatology Department, Université de Bordeaux, Bordeaux, France; EA2406, Université de Bordeaux, Bordeaux, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Beylot-Barry
authors.affiliation: Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France; APHP, Hôpital Saint-Louis, Dermatology Department, Paris, France.
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.affiliation: APHP, Hôpital Avicenne, Dermatology Department, Bobigny, France; Université Paris 13, Pôles de Recherche et d'Enseignement Supérieur (PRES) Sorbonne-Paris Cité, Paris, France. Electronic address: liliane.laroche@avc.aphp.fr.
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
authors.affiliation: Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Dermatology Department, Paris, France; Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Crickx
authors.affiliation: Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat-Claude-Bernard, Dermatology Department, Paris, France; Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France.
authors.forename: Eve
authors.initials: E
authors.lastname: Maubec
mesh_terms: D000287:Administration, Topical; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001707:Biopsy, Needle; D003131:Combined Modality Therapy; D003873:Dermatitis, Exfoliative; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D017893:Photopheresis / Q000379:methods; D035583:Rare Diseases; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012737:Sex Factors; D012751:Sezary Syndrome / Q000473:pathology* / Q000628:therapy*; D012878:Skin Neoplasms / Q000473:pathology* / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D008727:Methotrexate
keywords: Sézary cells; Sézary syndrome; chronic pruritus; erythroderma; invisible dermatosis; leukemic cutaneous T-cell lymphoma; nonerythrodermic; prognosis