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. (23rd June 2015)

• Record Type: Journal Article
• Authors: Klemke, C.D.; Booken, N.; Weiss, C.; Nicolay, J.P.; Goerdt, S.; Felcht, M.; Géraud, C.; Kempf, W.; Assaf, C.; Ortonne, N.; Battistella, M.; Bagot, M.; Knobler, R.; Quaglino, P.; Arheiliger, B.; Santucci, M.; Jansen, P.; Vermeer, M.H.; Willemze, R.
• Is Part Of: British journal of dermatology. Volume 173:Number 1(2015:Jul.)
• Page Start: 93
• Page End: 105
• Publication Date: 2015-06-23
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Periodicals; 616.5
• DOI: 10.1111/bjd.13832
• Languages: English
• ISSNs: 0007-0963

Abstract

<abstract abstract-type="main" id="bjd13832-abs-0001"> <title>Summary</title> <sec id="bjd13832-sec-0001" sec-type="section"> <title>Background</title> <p>Patients with erythrodermic disease are a diagnostic challenge regarding the clinical and histological differential diagnosis.</p> </sec> <sec id="bjd13832-sec-0002" sec-type="section"> <title>Objectives</title> <p>To evaluate histopathological and immunohistochemical diagnostic markers for Sézary syndrome.</p> </sec> <sec id="bjd13832-sec-0003" sec-type="section"> <title>Methods</title> <p>Ninety‐seven erythrodermic cases [Sézary syndrome (SS), <italic>n </italic>=<italic> </italic>57; erythrodermic inflammatory dermatoses (EIDs), <italic>n </italic>=<italic> </italic>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.</p> </sec> <sec id="bjd13832-sec-0004" sec-type="section"> <title>Results</title> <p>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 (<italic>P </italic><<italic> </italic>0·001) and more intraepidermal atypical lymphocytes (<italic>P </italic>=<italic> </italic>0·0014) in SS biopsies compared with EID. Pautrier microabscesses were seen only in SS (23%) and not in EID (<italic>P </italic>=<italic> </italic>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 (<italic>P </italic><<italic> </italic>0·001). The lymphocytic infiltrate in SS skin samples was found significantly to express PD‐1 (<italic>P </italic>=<italic> </italic>0·0053), MUM‐1 (<italic>P </italic>=<italic> </italic>0·0017) and Ki‐67 (<italic>P </italic><<italic> </italic>0·001), and showed less infiltration of CD8<sup>+</sup> lymphocytes (<italic>P </italic><<italic> </italic>0·001). A multivariate analysis identified CD7 loss, increased numbers of small cerebriform lymphocytes, low numbers of CD8<sup>+</sup> lymphocytes and increased proliferation (Ki‐67<sup>+</sup> lymphocytes) as the strongest indicators for the diagnosis of SS.</p> </sec> <sec id="bjd13832-sec-0005" sec-type="section"> <title>Conclusions</title> <p>A number of different histological and immunophenotypical criteria are required to differentiate between SS and EIDs.</p> </sec> </abstract>