Professional Documents
Culture Documents
1
Department of Dermatology, CHU de
Caen, Caen, France, 2Medical School,
de Caen Basse-Nomandie,
Universite
Caen, France, 3Department of
Haematology, CHU de Caen, Caen,
France, 4Department of Pathology, CHU de
Caen, Caen, France, and 5Department of
Pulmonary, CHU de Caen, Caen, France
Correspondence
Laurence Verneuil, MD, PHD
Dermatology Department, CHU Caen,
Avenue Georges Clemenceau, F-14033
Caen, France
E-mail: verneuil-l@chu-caen.fr
Conflicts of interest: None.
Introduction
Case report
81
82
Case report
Brugie re et al.
(a)
(b)
(c)
Discussion
Sezary syndrome characterized by a folliculotropism (folliculotropic Sezary syndrome) is exceptional with only eight
cases previously reported713 (Table 1). In our patient,
aggressiveness was remarkable with concomitant pulmonary lymphoma localization at the onset of disease. Currently, as in our case, a quick extracutaneous involvement
has been described in four of nine patients (44%) with folliculotropic Sezary syndrome, showing a strong aggressiveness when a folliculotropism is present7,8,13 (Table 1). This
fact suggests that factors other than therapeutic resistance
alone, which was recorded in folliculotropic MF,14,15 could
be involved in this agressiveness. The T lymphocytes found
in the folliculotropic form of CTCL could be characterized
by greater visceral tropism. This raises the question of the
molecular and functional characteristics of these T lymphocytes and the possibility of a common target in hair follicles
and certain organs. Studies have shown that chemokine
receptors are likely to be involved in the skin tropism that
characterizes CTCL.16,17 Molecular study characterizing
2014 The International Society of Dermatology
Brugie re et al.
5 months
At the diagnosis
Yes
Lung
At the diagnosis
Bone marrow
ND
ND, in life after 5 years
ND, in life after 2 years
In life
< 1 year
5 months
Bone marrow
Yes
Yes
Yes
Yes
2 years
ND
ND
5 months
Jang13
Mehta7
Gerami12
zary syndrome
Se
zary syndrome
Se
zary syndrome
Se
Cutaneous CD30+ folliculotropic
zary
lymphoma with circulating Se
cells
zary syndrome
Se
zary syndrome
Se
zary syndrome
Se
zary syndrome with
CD25+ Se
CD30+ large cell transformation
zary syndrome
Se
Westfried9
Rivers10
LeBoit11
Tremeau-Martinage8
M
M
F
M
Yes
No
Yes
Yes
Bone marrow
Survival
Sex
Type of folliculotropic lymphoma
Case report
Cases reported
Cutaneous data
Adenopathy
Extracutaneous
involvement
Time to visceral
involvement
Acknowledgements
We wish to thank A. Swaine, who reviewed the English
language.
References
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