Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients - 16/10/14

Doi : 10.1016/j.jaad.2014.06.033

Adèle de Masson, MD ^a,^c,^d, Maxime Battistella, MD ^b,^d,^e, Marie-Dominique Vignon-Pennamen, MD ^b, Bénédicte Cavelier-Balloy, MD ^b, Frédéric Mouly, MD ^a, Michel Rybojad, MD ^a, Jean-David Bouaziz, MD, PhD ^a,^c,^d, Antoine Petit, MD ^a, Anne Saussine, MD ^a, Sophie Ronceray, MD ^f, François Le Gall, MD ^f, Caroline Ram-Wolff, MD ^a, Philippe Assouly, MD ^a, Olivier Dereure, MD, PhD ^h, Pascal Joly, MD, PhD ⁱ, Alexiane Dallot, MD ^j, Alain Dupuy, MD, PhD ^g, Céleste Lebbé, MD, PhD ^a,^c,^d, Isabelle Moulonguet, MD ^a, Jacqueline Rivet, MD, PhD ^b, Anne Janin, MD, PhD ^b,^d,^e, Martine Bagot, MD, PhD ^a,^c,^d,^⁎
^a Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
^b Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
^c Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France
^d Université Paris VII Sorbonne Paris Cité, Paris, France
^e INSERM UMR-S928, Paris, France
^f Laboratoire de Pathologie, Hôpital Pontchaillou, Rennes, France
^g Service de Dermatologie, Hôpital Pontchaillou, Rennes, France
^h Service de Dermatologie, Hôpital Saint-Eloi, Montpellier, France
ⁱ Service de Dermatologie, Hôpital Charles-Nicolle, Rouen, France
^j Service de Dermatologie, Hôpital Robert-Ballanger, Aulnay, France

^∗Correspondence to: Martine Bagot, MD, PhD, Service de Dermatologie, Hôpital Saint-Louis, 1 avenue Claude Vellefaux, 75010, Paris, France.

Abstract

Background

A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the follicular subtype of MF in the World Health Organization–European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.

Objective

We sought to determine the clinicopathologic features and disease course of patients with STMF.

Methods

A retrospective study was conducted to identify patients with STMF from 1998 to 2013.

Results

Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n = 16, 84%) with associated alopecia (n = 12, 63%) and/or punctuated aspect (n = 12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P = .02).

Limitations

Retrospective setting is a limitation.

Conclusions

In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.

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Key words : cutaneous T-cell lymphoma, follicular, folliculotropism, mycosis fungoides, syringotropic, syringotropism

Abbreviations used : CI, DSS, EORTC, FMF, MF, OS, PCL, STMF, TCR, WHO

Plan

Methods

Results

Clinical features

Histopathologic, immunohistochemical, molecular features, and biological and radiologic parameters

	Funding sources: None.
	Conflicts of interest: None declared.
	Reprints not available from the authors.

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Vol 71 - N° 5

P. 926-934 - novembre 2014 Retour au numéro

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Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients - 16/10/14

Abstract

Background

Objective

Methods

Results

Limitations

Conclusions

Plan

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