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Review
. 2012 Nov-Dec;87(6):817-28; quiz 829-30.
doi: 10.1590/s0365-05962012000600001.

Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update

Thamy Yamashita  1 , Luciana Patricia Fernandes Abbade, Mariangela Esther Alencar Marques, Silvio Alencar Marques
Affiliations
Review

Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update

Thamy Yamashita et al. An Bras Dermatol. 2012 Nov-Dec.

Abstract
in English, Portuguese

This paper reviews the diagnostic and classificatory concepts of mycosis fungoides and Sézary syndrome in light of the latest normative publications. It describes the great variability of the clinical expression of mycosis fungoides in its early stages as well as the histopathological and immunohistochemical aspects that help with diagnosis. The diagnostic criteria required for characterizing Sézary syndrome and the staging system used for both mycosis fungoides and Sézary syndrome are described.

O artigo revisa os conceitos diagnósticos e de classificação da micose fungóide e da síndrome de Sézary a luz das publicações normativas mais recentes. Descreve a grande variabilidade de expressão clinica da micose fungóide em seus estágios iniciais assim como os aspectos histopatológicos e imuno-histoquímicos auxiliares ao diagnóstico. São descritos os critérios de diagnósticos exigidos para que se caracterize a síndrome de Sézary e o sistema de estadiamento, utilizado para ambas, micose fungóide e síndrome de Sézary.

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Conflict of interest statement

Conflict of interest: None

Figures

FIGURE 1
FIGURE 1
Mycosis fungoides - patch: erythematous, atrophic and slightly hyperkeratotic lesion on the gluteal region
FIGURE 2
FIGURE 2
Mycosis fungoides - patch: hypochromic and atrophic lesion on the thorax
FIGURE 3
FIGURE 3
Mycosis fungoides - plaque: erythemato-violaceous lesion with mild hyperkeratosis localized on the inguino-crural region
FIGURE 4
FIGURE 4
Mycosis fungoides: tumoral lesion appearing over a previous patch-type lesion on the leg
FIGURE 5
FIGURE 5
Mycosis fungoides: psoriasiform pattern with hyperplasia of the epidermis, band-like inflammatory infiltration and epidermotropism of lymphocytes. HE, (100X)
FIGURE 6
FIGURE 6
Mycosis fungoides: intense epidermotropism of lymphocytes on the epidermis and mild spongiosis. HE, (400X)
FIGURE 7
FIGURE 7
Mycosis fungoides: epidermotropism of lymphocytes and Pautrier’s micro-abscess. (HE X 400)
FIGURE 8
FIGURE 8
Mycosis fungoides: epidermotropism and lymphocyte alignment in the basal layer are shown. HE, (400X)
FIGURE 9
FIGURE 9
Mycosis fungoides: phenotypic studies showing CD3-positive T-cell on the epidermis and dermis. (CD3 immunostain 400X)
FIGURE 10
FIGURE 10
Mycosis fungoides: phenotypic studies showing CD4-positive T-cell on the epidermis and dermis. (CD4 immunostain 400X)
See this image and copyright information in PMC

References

    1. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785. - PubMed
    1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC; 2008.
    1. Rizyl MA, Evens MST, Nelson BP, Rosen ST. T-cell non-Hodgkin lymphoma. Blood. 2008;107:1255–1284. - PubMed
    1. Sanches JA, Jr, Moricz CZM, Neto CF. Processos linfoproliferativos da pele. Parte 2 - Linfomas cutâneos de células T e de células NK. An Bras Dermatol. 2006;81:7–25.
    1. Cerroni L, Gatter K, Kerl H, editors. Skin lymphoma. The illustrated guide. 3rd ed. Oxford, UK: Wiley-Blackwell; 2009. pp. 11–56. Chapter 2, Mycosis fungoides.

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