Sézary syndrome without erythroderma - 16/05/15

Doi : 10.1016/j.jaad.2014.11.015

Aurélia Henn, MD ^a, Laurence Michel, PhD ^b, Charlotte Fite, MD ^a,^c, Lydia Deschamps, MD ^c,^d, Nicolas Ortonne, MD, PhD ^e,^f, Saskia Ingen-Housz-Oro, MD ^g, Eduardo Marinho, MD ^c,^d, Marie Beylot-Barry, MD, PhD ^h,ⁱ, Martine Bagot, MD, PhD ^c,^j, Liliane Laroche, MD, PhD ^k,^l,^⁎ , Béatrice Crickx, MD, PhD ^a,^c, Eve Maubec, MD, PhD ^a,^c
^a Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat–Claude-Bernard, Dermatology Department, Paris, France
^d Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Bichat–Claude-Bernard, Pathology Department, Paris, France
^b Institut National de la Santé et de la Recherche Médicale (INSERM) U976, Hôpital Saint-Louis, Paris, France
^c Université Paris 7-Denis Diderot, PRES Sorbonne-Paris Cité, Paris, France
^e APHP, Groupe Hospitalier Henri-Mondor, Pathology Department, Créteil, France
^g APHP, Groupe Hospitalier Henri-Mondor, Dermatology Department, Créteil, France
^f Université Paris Est-Créteil, Créteil, France
^h Centre Hospitalo-Universitaire (CHU) de Bordeaux, Dermatology Department, Université de Bordeaux, Bordeaux, France
ⁱ EA2406, Université de Bordeaux, Bordeaux, France
^j APHP, Hôpital Saint-Louis, Dermatology Department, Paris, France
^k APHP, Hôpital Avicenne, Dermatology Department, Bobigny, France
^l Université Paris 13, Pôles de Recherche et d'Enseignement Supérieur (PRES) Sorbonne-Paris Cité, Paris, France

^∗Reprint requests: Liliane Laroche, MD, PhD, Hôpital Avicenne, Service de Dermatologie, 125, route de Stalingrad, 93009 Bobigny Cedex, France.

Abstract

Background

Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement.

Objective

We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma.

Methods

Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied.

Results

Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred.

Limitations

Retrospective design and small sample size are limitations.

Conclusion

Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.

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Key words : chronic pruritus, erythroderma, invisible dermatosis, leukemic cutaneous T-cell lymphoma, nonerythrodermic, prognosis, Sézary cells, Sézary syndrome

Abbreviations used : CTCL, EORTC, ISCL, MF, PBL, PCR, PD, TCR

Plan

	Drs Michel and Fite contributed equally to this work, as did Drs Laroche, Crickx, and Maubec.
	Funding sources: None.
	Conflicts of interest: None declared.

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Vol 72 - N° 6

P. 1003 - juin 2015 Retour au numéro

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Sézary syndrome without erythroderma - 16/05/15

Abstract

Background

Objective

Methods

Results

Limitations

Conclusion

Plan

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