C D Klemke1, N Booken1, C Weiss2, J P Nicolay1, S Goerdt1, M Felcht1, C Géraud1, W Kempf3, C Assaf4, N Ortonne5, M Battistella6, M Bagot7, R Knobler8, P Quaglino9, B Arheiliger10, M Santucci11, P Jansen12, M H Vermeer12, R Willemze12. 1. Department of Dermatology, Venereology and Allergology, University Medical Center Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. 2. Division of Statistics, University Medical Center Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. 3. Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland. 4. Department of Dermatology, Helios Clinic Krefeld, Krefeld, Germany. 5. Department of Pathology, Hôpital Henri-Mondor, Créteil, France. 6. Department of Pathology, AP-HP, Hôpital Saint Louis, Université Paris Diderot, Sorbonne Paris Cité, UMR-S 1165, F-75010, Paris, France. 7. Department of Dermatology, Hôpital Saint-Louis, Université Paris Diderot, Sorbonne Paris Cité, Inserm U976, 1 Avenue Claude Vellefaux, 75010, Paris, France. 8. Department of Dermatology, University of Vienna, Vienna, Austria. 9. Dermatologic Clinic, Department of Medical Science, University of Torino, Torino, Italy. 10. Department of Dermatology, Johannes Wesling Klinikum, Minden, Germany. 11. Division of Pathological Anatomy, University of Florence, Florence, Italy. 12. Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands.
Abstract
BACKGROUND: Patients with erythrodermic disease are a diagnostic challenge regarding the clinical and histological differential diagnosis. OBJECTIVES: To evaluate histopathological and immunohistochemical diagnostic markers for Sézary syndrome. METHODS: Ninety-seven erythrodermic cases [Sézary syndrome (SS), n = 57; erythrodermic inflammatory dermatoses (EIDs), n = 40] were collected by the EORTC Cutaneous Lymphoma Task Force histopathology group. Evaluation criteria were (i) epidermal and dermal changes; (ii) morphology of the infiltrate; (iii) immunohistochemical analysis of marker loss (CD2, CD3, CD4, CD5 and CD7); (iv) bystander infiltrate by staining for CD8, FOXP3 and CD25; and (v) expression of Ki-67, CD30, PD-1 and MUM-1. RESULTS: The workshop panel made a correct diagnosis of SS in 51% of cases (cutaneous T-cell lymphoma 81%) and of EID in 80% without clinical or laboratory data. Histology revealed a significantly increased degree of epidermotropism (P < 0.001) and more intraepidermal atypical lymphocytes (P = 0.0014) in SS biopsies compared with EID. Pautrier microabscesses were seen only in SS (23%) and not in EID (P = 0.0012). SS showed significantly more dermal cerebriform and blastic lymphocytes than EID. Immunohistochemistry revealed a significant loss of CD7 expression (< 50%) in 33 of 51 (65%) cases of SS compared with two of 35 (6%) EID (P < 0.001). The lymphocytic infiltrate in SS skin samples was found significantly to express PD-1 (P = 0.0053), MUM-1 (P = 0.0017) and Ki-67 (P < 0.001), and showed less infiltration of CD8(+) lymphocytes (P < 0.001). A multivariate analysis identified CD7 loss, increased numbers of small cerebriform lymphocytes, low numbers of CD8(+) lymphocytes and increased proliferation (Ki-67(+) lymphocytes) as the strongest indicators for the diagnosis of SS. CONCLUSIONS: A number of different histological and immunophenotypical criteria are required to differentiate between SS and EIDs.
BACKGROUND:Patients with erythrodermic disease are a diagnostic challenge regarding the clinical and histological differential diagnosis. OBJECTIVES: To evaluate histopathological and immunohistochemical diagnostic markers for Sézary syndrome. METHODS: Ninety-seven erythrodermic cases [Sézary syndrome (SS), n = 57; erythrodermic inflammatory dermatoses (EIDs), n = 40] were collected by the EORTC Cutaneous Lymphoma Task Force histopathology group. Evaluation criteria were (i) epidermal and dermal changes; (ii) morphology of the infiltrate; (iii) immunohistochemical analysis of marker loss (CD2, CD3, CD4, CD5 and CD7); (iv) bystander infiltrate by staining for CD8, FOXP3 and CD25; and (v) expression of Ki-67, CD30, PD-1 and MUM-1. RESULTS: The workshop panel made a correct diagnosis of SS in 51% of cases (cutaneous T-cell lymphoma 81%) and of EID in 80% without clinical or laboratory data. Histology revealed a significantly increased degree of epidermotropism (P < 0.001) and more intraepidermal atypical lymphocytes (P = 0.0014) in SS biopsies compared with EID. Pautrier microabscesses were seen only in SS (23%) and not in EID (P = 0.0012). SS showed significantly more dermal cerebriform and blastic lymphocytes than EID. Immunohistochemistry revealed a significant loss of CD7 expression (< 50%) in 33 of 51 (65%) cases of SS compared with two of 35 (6%) EID (P < 0.001). The lymphocytic infiltrate in SS skin samples was found significantly to express PD-1 (P = 0.0053), MUM-1 (P = 0.0017) and Ki-67 (P < 0.001), and showed less infiltration of CD8(+) lymphocytes (P < 0.001). A multivariate analysis identified CD7 loss, increased numbers of small cerebriform lymphocytes, low numbers of CD8(+) lymphocytes and increased proliferation (Ki-67(+) lymphocytes) as the strongest indicators for the diagnosis of SS. CONCLUSIONS: A number of different histological and immunophenotypical criteria are required to differentiate between SS and EIDs.
Authors: Julia J Scarisbrick; H Miles Prince; Maarten H Vermeer; Pietro Quaglino; Steven Horwitz; Pierluigi Porcu; Rudolf Stadler; Gary S Wood; Marie Beylot-Barry; Anne Pham-Ledard; Francine Foss; Michael Girardi; Martine Bagot; Laurence Michel; Maxime Battistella; Joan Guitart; Timothy M Kuzel; Maria Estela Martinez-Escala; Teresa Estrach; Evangelia Papadavid; Christina Antoniou; Dimitis Rigopoulos; Vassilki Nikolaou; Makoto Sugaya; Tomomitsu Miyagaki; Robert Gniadecki; José Antonio Sanches; Jade Cury-Martins; Denis Miyashiro; Octavio Servitje; Cristina Muniesa; Emilio Berti; Francesco Onida; Laura Corti; Emilia Hodak; Iris Amitay-Laish; Pablo L Ortiz-Romero; Jose L Rodríguez-Peralto; Robert Knobler; Stefanie Porkert; Wolfgang Bauer; Nicola Pimpinelli; Vieri Grandi; Richard Cowan; Alain Rook; Ellen Kim; Alessandro Pileri; Annalisa Patrizi; Ramon M Pujol; Henry Wong; Kelly Tyler; Rene Stranzenbach; Christiane Querfeld; Paolo Fava; Milena Maule; Rein Willemze; Felicity Evison; Stephen Morris; Robert Twigger; Rakhshandra Talpur; Jinah Kim; Grant Ognibene; Shufeng Li; Mahkam Tavallaee; Richard T Hoppe; Madeleine Duvic; Sean J Whittaker; Youn H Kim Journal: J Clin Oncol Date: 2015-10-05 Impact factor: 44.544
Authors: Rein Willemze; Lorenzo Cerroni; Werner Kempf; Emilio Berti; Fabio Facchetti; Steven H Swerdlow; Elaine S Jaffe Journal: Blood Date: 2019-01-11 Impact factor: 22.113