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Unusual association of diseases/symptoms
A 27-year-old man with diplopia, fatiguable ptosis and
rash: a rare presentation of angiocentric T cell lymphoma
with lymphomatoid vasculitis
Stela Lefter,
1
Patrick Forde,
2
Hebah Nashat,
3
Eugene Moylan,
2
Yaseen Yacoob
1
1
Department of Neurology, Cork University Hospital, Cork, Ireland
2
Department of Oncology, Cork University Hospital, Cork, Ireland
3
Department of Dermatology, Cork University Hospital, Cork, Ireland
Correspondence to Stela Lefter, stela_lefter@yahoo.com
Summary
A 27-year-old man presented with a 36-h history of ptosis and diplopia and a 10-day history of a lower limb rash. Skin biopsy revealed an aggres-
sive angiocentric γδ-T cell lymphoma. The patient’s symptoms and signs disappeared within 1 week of commencement of chemotherapy and
there are plans for allogeneic stem cell transplantation.
BACKGROUND
Primary cutaneous γδ-T cell lymphoma is a rare aggressive
variant of peripheral T cell lymphomas with only 40 cases
described in the literature.
1–9
There are few reported cases
of angiocentric lymphoma involving the cranial nerves.
This case report is important from many viewpoints: pri-
mary cutaneous angiocentric γδ-lymphoma is a rare lym-
phoma; skin biopsy histology revealed evidence of an
infiltrating neoplastic lymphomatoid vasculitis; this same
process more than likely caused perineural vascular inflam-
mation resulting in the neurological presentation; and the
patient actually presented with ptosis and diplopia. Our
case is rare and instructive in that our patient had both cuta-
neous and neurologic signs at presentation.
CASE PRESENTATION
A previously healthy 27-year-old man presented with a
36-h history of ptosis, diplopia and a 10-day history of a
sparse predominantly lower limb rash. The patient woke up
the day before admission with a feeling of pressure behind
his right eye. This was followed the next day by right-sided
ptosis. He complained of double vision on looking to the
right and upwards and a transient burning sensation of the
right side of his face and right ear. He described feeling
dizzy when walking but reported no problems with speech,
swallowing or muscle weakness. Further full review of sys-
tems was negative (eg, no fever, pruritus, weight loss or
sweating). Social and family history were non-contributory.
Physical examination did not reveal lymphadenopathy or
organomegaly. Examination revealed bilateral fatiguable
ptosis with normal eye movements but diplopia on right
lateral and upward gaze. There was no pupillary involve-
ment. There was no fatiguable bulbar/trunk/limb muscular
weakness or focal lateralising signs. He had vasculitic-type
skin lesions below the knees bilaterally with one lesion on
each forearm (figure 1).
INVESTIGATIONS
Full blood count (FBC), differential white cell count and all
blood/urine tests were normal. Serologic and blood workup
for infectious diseases, connective tissue diseases and sys-
temic vasculitis were negative. CT of thorax/abdomen/
pelvis, MRI of the brain with gadolinium, tensilon test,
single-fibre electromyography/repetitive nerve stimulation,
and antiacetylcholine receptor, antimuscle-specific-kinase,
antivoltage-gated-calcium-channel and paraneoplastic anti-
bodies were negative. Cerebrospinal fluid analysis includ-
ing cytology was normal. Lower limb skin biopsy (figure 2)
revealed a very rare primary cutaneous angiocentric T cell
lymphoma (γδ variant) with lymphomatoid vasculitis.
Immunohistochemical staining of the lymphocytes was
strongly positive for T cell markers, CD2, CD7, CD3 and
CD43, but negative for B cell markers, CD20 and CD79a.
There was double negativity for CD4 and CD8. This com-
bination of histological and immunohistochemical findings
is highly suggestive of an angiocentric γδ variant of T cell
lymphoma with associated lymphomatoid vasculitis. Bone
marrow biopsy confirmed lymphomatous involvement.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis included myasthenia gravis, a
systemic vasculitis, infectious causes, inflammatory causes
and neoplastic/paraneoplastic processes.
TREATMENT
Hyper-CVAD chemotherapy (ie, cyclophosphamide, vinc-
ristine, adriamycin, dexamethasone) was commenced.
OUTCOME AND FOLLOW-UP
Within 1 week of commencing chemotherapy, diplopia,
ptosis and skin lesions had resolved completely. Chemo-
therapy is ongoing with plans for an allogeneic stem cell
transplantation.
1of3BMJ Case Reports 2010; doi:10.1136/bcr.06.2010.3118
DISCUSSION
Peripheral T cell lymphomas are uncommon neoplasms and
account for approximately 12% of all lymphoid tumours
worldwide.
9
Primary cutaneous γδ-T cell lymphoma is a
rare aggressive variant of peripheral T cell lymphomas with
at most no more than 45 cases described in the literature.
49
This variant accounts for less than 1% of all cutaneous T cell
lymphomas with a median age of presentation of 54 years
(range 13–82 years) and a male:female ratio of 16:7.
79
There
are few reported cases of angiocentric lymphoma involving
Figure 1 Right ankle vasculitic-type skin lesions.
Figure 2 Skin biopsy (ankle lesion) showing lymphomatoid infiltrative process (bottom blue arrow), a thrombosed vessel (top blue arrow)
and necrosis (yellow arrow).
2of3 BMJ Case Reports 2010; doi:10.1136/bcr.06.2010.3118
the cranial nerves.
1–3
Two distinct γδ-T cell lymphoma enti-
ties are recognised: hepatosplenic T cell lymphoma and pri-
mary cutaneous γδ-T cell lymphoma as seen in this case.
Angiocentric T cell lymphoma may present with a broad
variety of cutaneous manifestations including papules, nod-
ules and ulcerated tumours and may masquerade as a cuta-
neous vasculitis both clinically and pathologically.
5
The
skin biopsy showed an infiltrating lymphomatoid vasculitis
causing perineural vascular inflammation. A likely explana-
tion for this patient’s presentation is that the same lym-
phomatoid vasculitic pathology occurred in his cranial
nerves with direct perineural involvement of the superior
divisions of both third nerves (preferentially affecting some
fascicles) and other cranial nerves as well, that is, right-sided
sixth and fifth nerves (the latter causing facial dysaesthe-
sia).
136
The prognosis of patients with angiocentric T cell
lymphoma is poor with reported median survival of 15
months.
78
Learning points
▲
Clinicians should remain cognizant of the fact that rare
T cell lymphomas can present without a history of
fever, weight loss or sweating.
▲
Cutaneous T cell lymphomas can present with a
normal FBC and differential white cell count.
▲
Cutaneous T cell lymphomas can present without
lymphadenopathy or organomegaly.
▲
Although this patient’s reason for presenting was
neurologic, the diagnosis was made by a skin biopsy of
lesions found by the admitting doctor on routine
admitting physical examination. An important take
home message here is that clinicians should always
carry out a full physical examination when admitting/
assessing patients.
▲
Fatiguableptosis doesnotalways indicatemyasthenia
gravis.Clinicalcontext isthe key.Even beforeworkup for
suchaneuromuscular disorderis done,thesymptoms
andsignscould pointtowards analternatediagnosis (eg,
theskinrash wasa pivotalfindingin thiscase).
Competing interests None.
Patient consent Obtained.
REFERENCES
1. Brazis PW, Menke DM, McLeish WM, et al. Angiocentric T-cell lymphoma
presenting with multiple cranial nerve palsies and retrobulbar optic
neuropathy. J Neuroophthalmol 1995;15:152–7.
2. Larner AJ. Proximal superior division oculomotor nerve palsy from
metastatic subarachnoid infiltration. J Neurol 2002;249:343–4.
3. Celebisoy N, Celebisoy M, Tokuçoglu F, et al. Superior division paresis of the
oculomotor nerve: Report of four cases. Eur Neurol 2006;56:50–3.
4. Koch R, Jaffe ES, Mensing C, et al. Cutaneous gamma/delta T-cell
lymphoma. J Dtsch Dermatol Ges 2009;7:1065–7.
5. Thomas R, Vuitch F, Lakhanpal S. Angiocentric T cell lymphoma
masquerading as cutaneous vasculitis. J Rheumatol 1994;21:760–2.
6. Cho K, Kim C, Yang S, et al. Angiocentric T cell lymphoma of the skin
presenting as inflammatory nodules of the leg. Clin Exp Dermatol
1997;22:104–8.
7. Toro JR, Beaty M, Sorbara L, et al. Gamma delta T-cell lymphoma of the
skin: a clinical, microscopic, and molecular study. Arch Dermatol
2000;136:1024–32.
8. Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is
associated with significantly decreased survival in cutaneous T-cell
lymphoma. Blood 2003;101:3407–12.
9. Tripodo C, Iannitto E, Florena AM, et al. Gamma-delta T-cell lymphomas. Nat
Rev Clin Oncol 2009;6:707–17.
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Lefter S, Forde P, Nashat H, Moylan E, Yacoob Y. A 27-year-old man with diplopia, fatiguable ptosis and rash: a rare presentation of angiocentric T cell lymphoma
with lymphomatoid vasculitis. BMJ Case Reports 2010;10.1136/bcr.06.2010.3118, date of publication
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