Acta Derm Venereol 89
Letters to the Editor
© 2009 The Authors. doi: 10.2340/00015555-0671
Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL)
is an uncommon type of peripheral T-cell lymphoma. The
classical clinical presentation is erythematous, subcuta-
neous plaques or nodules involving the extremities and
the trunk. Two distinctive entities are described: SPTCL
with α/β or γ/δ phenotype. We report here an atypical
clinical presentation of SPTCL, illustrating the difficul-
ties of diagnosis of this kind of T-cell lymphoma.
A 54-year-old man, with no medical history, was referred for
evaluation of an erythematous, oedematous subcutaneous
infiltration of the trunk. The symptoms had begun 2 months
earlier with numerous, tender, erythematous nodules of the
trunk, associated with fever up to 39°C that persisted despite
antibiotics. Very quickly, the cutaneous symptoms changed
into an erythematous, oedematous thickening of the skin of
the trunk with no visible nodules (Fig. 1). This was associated
with a weight gain of 10 kg. No lymphadenopathy was found.
Laboratory studies revealed anaemia (haemoglobin: 10 g/dl), ele-
vated liver function test findings (serum glutamate oxaloacetate
transaminase: 156 UI/l; serum glutamate pyruvate transaminase:
128 UI/l; normal value < 30 UI/l), elevated lactic dehydroge-
nase (LDH) 1707 UI/l (normal < 420 UI/l), hyperferritinaemia
13,946 µg/l (normal < 80 µg/l) and hypertriglyceridaemia 5.61 g/l
(normal < 1.7g/l). Immunological and bacteriological labora-
tory studies and viral serologies were all negative. Several
punch biopsies were performed. In each of these the epidermis
showed reactive changes, with no epidermotropism, associated
with a mild dermal inflammatory infiltrate simulating pannicu-
litis. However, the biopsies were too superficial to conclude. A
thoracic/abdominal computed tomography (CT) scan showed a
diffused subcutaneous thoracic and abdominal wall infiltration,
hepatosplenomegaly without deep lymphadenopathy (Fig. 2a).
Bone marrow biopsy did not show haemophagocytosis or
involve ment by lymphoma.
A deep incisional biopsy specimen of the abdominal infiltra-
tion was performed. It displayed aggregates of foamy histiocytes
and lymphocytes extending into subcutaneous fat associated
with necrosis and karryorrhexis (Fig. 3a). The infiltrate was
mild and consisted of small, medium and large lymphoid cells
with irregular nuclear contour and densely clumped chromatin.
These atypical lymphocytes were rimming fat lobules.
Immunophenotypical analysis in paraffin sections showed that
the neoplastic cells were of T-cell phenotype, stained with CD45,
CD3, and CD8, but CD56- and CD30-negative. In addition, there
was CD7 antigen loss. CD68+ staining revealed the presence
of histiocytes. Molecular biological studies (polymerase chain
reaction (PCR)) of α/β T cell receptor (TCR) in the skin and the
blood revealed clonal T-cell population. Immunohistochemistry
studies using anti-human α/β TCR and anti-human γ/δ TCR
(mouse IgG1k, Ebioscience, Montrouge, France) confirmed that
TCR rearrangement was α/β+ (Fig. 3b).
Thus, a diagnosis of α/β SPTCL was made. Despite normal bone
marrow, clinical and biological symptoms (fever, hepatosplenome-
galy, cytopaenia, hyperferritinaemia and hypertryglyceridaemia)
suggested an association with haemophagocytic syndrome.
The patient was treated with eight cycles of CHOP chemotherapy
(cyclophosphamide (750 mg/m2 day 1), doxorubicin (50 mg/m2
day 1), vincristine (1.4 mg/m2 day 1) and prednisone (80 mg/m2
per day 1–5)) with partial clinical remission. A CT scan performed
after eight cycles of CHOP revealed persistent mild subcutaneous
infiltration, confirmed by positron emisson tomography (PET)
scan, but hepatosplenomegaly had regressed to normal size (Fig.
2b). Biological abnormalities were almost corrected, with only
persistent elevated LDH (1.5 N). Due to borderline persistent
thrombopaenia (82 × 109/l without sign of relapse: normal bone
marrow and negative analysis for T-cell receptor gene rearrange-
ment), CHOP chemotherapy was stopped and autologous stem
Thoracic Subcutaneous Infiltration: An Unusual Presentation of Subcutaneous Panniculitis-like
Fabienne Ballanger1, Sébastien Barbarot1, Steven Le Gouill2, Fanny Gaillard3, Elisabeth Cassagnau3, Laurence Lodé4, Brigitte
Dréno1 and Jean F. Stalder1
Departments of 1Dermatology, 2Haematology, 3Anatomopathology and 4Molecular Biology, Hôtel Dieu, FR-44035 Nantes, France. E-mail: fabienne.
Accepted March 12, 2009.
Fig. 2. Computed tomography scan (left) before and (right) after chemotherapy:
subcutaneous infiltration of the trunk and diminution of the subcutaneous
Fig. 1. Clinical presentation of subcutaneous panniculitis-like T-cell lymphoma
as diffuse thoracic subcutaneous infiltration.
Letters to the Editor
cell transplantation was proposed, but was refused by the patient.
Maintenance chemotherapy with NIPENT (pentostatin 4 mg/m2
every 3 weeks) and dexamethasone was commenced. Twelve
cycles were realized, with good biological and clinical tolerance.
At the present time, clinical and radiological evaluation shows
that the patient has remained in a stable condition. The follow-up
duration after obtaining partial remission is 10 months since the
end of the chemotherapy.
SPTCL is a rare primary cutaneous T-cell lymphoma,
first described by Gonzales et al. in 1991 (1). It is cur-
rently classified in the category of peripheral T-cell
neoplasms (2). The median age at diagnosis is approx-
imately 39 years. The classical clinical presentation
consists of tender erythematous nodules, and the lower
extremities are the most frequent sites of cutaneous
involvement, followed by the trunk, arms and face.
Ulceration occurs in 20% of cases (3). Biologically,
cytopaenia occurs in approximately 20% of cases and
liver dysfunction, as presented by our patient, occurs
in 10%. Histologically, SPTCL is characterized by
lobular or mixed septal and lobular panniculitis, often
combined with cytophagocytosis. Malignant lympho-
cytes are small to medium size with cytological atypia,
which may be minimal, as in our case. This explains
why it is frequently misdiagnosed as a benign inflam-
matory disorder. Typically, lymphocytes infiltrate the
fat in a lobular lace-like pattern, rimming the fat spaces,
and are associated with karryorrhexis. Immunohisto-
chemical staining is positive for T-cell antigen. CD7
antigen loss is observed in 30% of patients (4). Clonal
rearrangement of TCR, shown by PCR, confirms T-cell
origin and clonality in 85% of cases.
In recent years, it has been observed that although
SPTCL with α/β phenotype has an excellent prognosis,
in particular when not complicated by haemophagocytic
syndrome (HPS), a rapid clinical deterioration secondary
to HPS is frequent in SPTCL with γ/δ phenotype (3).
HPS is a multisystem illness characterized by fever,
wasting, adenopathy, hepatosplenomegaly, pancyto-
paenia, coagulopathy, hyperferritinaemia and hyper-
triglyceridaemia. It develops in 45–70% of patients
with SPTCL, with a mortality rate of 81% (4, 5). HPS
associated with panniculitis has even been reported
without any clear evidence of malignant lymphoma (4,
6). HPS, cytopaenia, and elevated LDH appear to be
poor prognostic factors (7–9).
The standard treatment of the disease has not yet been
established. As SPTCL with α/β phenotype and SPTCL
with γ/δ phenotype appear to be two distinct entities with
different prognoses, treatment should be adapted (2).
Patients presenting with α/β TCR+SPTCL without HPS
may benefit equally either from prednisone or others im-
munosuppressive agents. In the case of a solitary lesion,
radiotherapy may be considered. However, low-dose
chemotherapeutic regimens (e.g. CHOP), sometimes
associated with local radiotherapy, may be efficient (10).
In the case of γ/δ SPTCL, early initiation of aggressive
chemotherapy followed by stem-cell transplantation
is recommended for better clinical outcome (2). In
one case, cyclosporine has been of benefit in SCPTL
refractory to CHOP (11). More recently, Hathaway
et al. (12) have reported the efficiency of denileukin
diftitox treatment in association with corticosteroids
(12). In our case, due to the presence of HPS, it was
rapidly decided to use multi-agent chemotherapy. This
could explain the partial clinical response even though
the patient presented with few poor prognostic factors.
However, stem-cell transplantation was refused by our
patient and pentostatin was chosen for maintenance
treatment. Pentostatin is a potent inhibitor of adenosine
deaminase and is selectively toxic to lymphocytes. It
has been used in human γ/δ+ T-cell malignancies (13)
and in cutaneous T-cell lymphoma, especially Sézary
syndrome (14, 15).
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Immunohistochemistry using anti-human
α/β T-cell receptor antibody (×400).
Acta Derm Venereol 89
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Acta Derm Venereol 89