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| The British journal of dermatology. 2015 Jun 23. doi: 10.1111/bjd.13832 |
| 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. |
| Klemke CD1, Booken N2, Weiss C3, Nicolay JP4, Goerdt S5, Felcht M6, Géraud C7, Kempf W8, Assaf C9, Ortonne N10, Battistella M11, Bagot M12, Knobler R13, Quaglino P14, Arheiliger B15, Santucci M16, Jansen P17, Vermeer MH18, Willemze R19 |
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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. |
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© 2015 British Association of Dermatologists. |
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Publikations ID: 25864856 Quelle: öffnen |
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