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Histopathological and immunophenotypical criteria for the diagnosis of Sézary syndrome in differentiation from other erythrodermic skin diseases: a European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force Study of 97 cases

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 syn...

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Main Authors: Klemke, Claus-Detlev (Author) , Booken, Nina (Author) , Weiß, Christel (Author) , Nicolay, Jan Peter (Author) , Goerdt, Sergij (Author) , Felcht, Moritz (Author) , Géraud, Cyrill (Author)
Format: Article (Journal)
Language:English
Published: July 2015
In: British journal of dermatology
Year: 2015, Volume: 173, Issue: 1, Pages: 93-105
ISSN:1365-2133
DOI:10.1111/bjd.13832
Online Access:Verlag, Volltext: http://dx.doi.org/10.1111/bjd.13832
Verlag, Volltext: http://onlinelibrary.wiley.com/doi/10.1111/bjd.13832/abstract
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Author Notes:C.D. Klemke, N. Booken, C. Weiss, J.P. Nicolay, S. Goerdt, M. Felcht, C. Géraud, W. Kempf, C. Assaf, N. Ortonne, M. Battistella, M. Bagot, R. Knobler, P. Quaglino, B. Arheiliger, M. Santucci, P. Jansen, M.H. Vermeer and R. Willemze
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Summary: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.
Item Description:Gesehen am 24.01.2018
Physical Description:Online Resource
ISSN:1365-2133
DOI:10.1111/bjd.13832

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