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Case report

zary syndrome with concomitant pulmonary


An unusual Se
localization: aggressiveness of folliculotropic form
Charlotte Brugiere1,2, MD, Andrea Stefan1,2, MD, Veronique Salaun3, MD, Francois
Comoz4, MD, Karine Campbell5, MD, Sylvain Chantepie3, MD, and Laurence Verneuil1,2,
MD, PHD

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

Cutaneous T-cell lymphomas (CTCL) are a heterogeneous


group of non-Hodgkin lymphomas arising from T cells
that home to and persist in the skin.1,2 Mycosis fungoides
(MF) and Sezary syndrome account for approximately
65% of CTCL.3 They were considered as different stages
in the same disease, but recent genetic and phenotypic
studies suggest that they arise from two distinct T-cell
subsets.4,5
Folliculotropic MF, a follicular variant of classic MF, is
discussed separately because of its distinct clinicopathological features3 and a more aggressive clinical course,
with poorer prognosis than in classic MF.6 It is characterized by folliculotropic infiltrates, often sparing the epidermis, and clinically by follicular papules, comedones, cysts
of preferentially head and neck areas, or by alopecia. It is
frequently refractory to usual therapies.6
Sezary syndrome is an aggressive clinical entity
associated with poor prognosis and a median survival of
23 years. Folliculotropic Sezary syndrome, a clinical variant, is exceptional, and we report a case of folliculotropic Sezary syndrome with a concomitant pulmonary
localization.

We report a 64-year-old man with a 1-month history


of pruritic erythroderma, which covered more than
80% of the body surface area and was associated with
infiltrated leonine facies, follicular papules of the head
and neck, alopecia of the scalp (Fig. 1a), and palmoplantar keratoderma. Cervical, axillary, and inguinal
lymphadenopathy were detected. Skin biopsy showed
no epidermotropism but follicular mucinosis was
confirmed by alcian blue staining (Fig. 1b) and lymphocyte aggregates composed of small- to intermediate-sized
lymphocytes with irregular nuclei (Fig. 1c) expressing
CD3/CD4/CD5 with a loss of CD7 and CD8 around
the hair follicles. An abnormal cutaneous T-cell population expressing CD2/CD3/CD4/CD5 with a loss of CD7
and CD26 expression was evidenced using flow cytometric immunophenotyping. Inguinal lymphadenectomy
showed localization of a malignant lymphoproliferative
T-cell population.
In the blood and inguinal lymphadenectomy, using
flow-cytometric immunophenotyping, the same T-cell
population was detected. The white blood cell count
showed 6.0 9 109/l lymphocytes with 1200/mm3 Sezary

2014 The International Society of Dermatology

International Journal of Dermatology 2016, 55, 8184

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82

Case report

Brugie re et al.

An unusual folliculotropic Sezary syndrome

(a)

(b)

(c)

Figure 1 Clinical and histological data


of our patient. (a) Infiltrated leonine
facies with alopecia of the scalp and
follicular papules of the head. (b)
Follicular mucinosis and aggregates of
lymphocytes around hair follicles;
alcian blue staining, 9 200. (c) Smallto intermediate-sized lymphocytes
with irregular nuclei at the lower part
of the hair follicle bulb; hematoxylin
eosinsafran staining, 9 400

cells and a CD4/CD8 ratio at 41. HTLV-1 detection was


negative.
A computed tomography scan showed bilateral reticular infiltrates, and bronchoalveolar fluid analyses, using
flow-cytometric immunophenotyping, detected the same
abnormal T-cell population CD2+/CD3+/CD4+/CD5+/
CD7 /CD8 /CD26 as in the skin, blood, and lymph
node. No red blood cell was detected in the bronchoalveolar fluid, excluding blood contamination. The same
T-cell clone was detected in the skin, blood, lymph nodes,
and bronchoalveolar fluid.
These data were characteristic of a folliculotropic
Sezary syndrome with a concomitant pulmonary localization, corresponding to a T4N2M1B2 stage, clinical
stage IVB. There was no other extracutaneous infiltration.
Despite intensive treatment with cyclophosphamide/
doxorubicin/vincristine/prednisone started immediately in
our patient, he died five months after the diagnosis due
to an acute respiratory distress syndrome probably secondary to the progression of pulmonary lymphoma.
International Journal of Dermatology 2016, 55, 8184

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

Follicular dermatosis, nodules, alopecia


Erythroderma, follicular papules, leonine facies
Erythroderma, follicular papules, alopecia
Erythroderma, follicular papules, leonine facies,
keratoderma
Erythroderma, follicular papules, leonine facies,
keratoderma, alopecia
F
M
M
M

2 years
ND
ND
5 months

Our case, 2012

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

Follicular papules, keratoderma


Erythroderma, nodules, small milia, alopecia
Erythema, follicular papules, alopecia
Follicular papules

Yes
No
Yes
Yes

Bone marrow

Survival
Sex
Type of folliculotropic lymphoma

2014 The International Society of Dermatology

Case report

and comparing T lymphocytes of folliculotropic CTCL and


T lymphocytes of non-folliculotropic CTCL could be
investigated.

Cases reported

Table 1 Reported cases of folliculotropic S


ezary syndrome

Cutaneous data

Adenopathy

Extracutaneous
involvement

Time to visceral
involvement

An unusual folliculotropic Sezary syndrome

Acknowledgements
We wish to thank A. Swaine, who reviewed the English
language.
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2014 The International Society of Dermatology