Actinic reticuloid-like CTCL 
Case report 
ACTINIC RETICULOID-LIKE CUTANEOUS T CELL 
LYMPHOMA 
Petrič G .. Lamovec L Kansky A. 
ABSTRACT 
According to textbooks mycosis fungoides (MF), Sezary syndrome (SS) and various cutaneous T cell lymphomas (CTCL) 
are clearly defined nosologic entities. Sometimes cases are encountered which are difficult to assign to one of the above 
named entities. The case of a 52-year old patient is described with cutaneous manifestations closely resembling the Sezary 
syndrome with traits of photosensitivity, with malignant cells characteristic of a lymphoma of high grade of malignancy in 
the lymph nodes and in the skin. but without Lutzner cells in peripheral blood. 
Treatrnent with chemotherapeutics Cyclophosphamid. Adrioblastin and Vincristin, Bleomycin, Oncovin resulted in an only 
limited success. while the introduction of methotrexate 180 mg once weekly proved to be very efficient. 
KEYWORDS 
cutaneous T cell lymphoma, actinic reticuloid-like, Sezary syndrome-like, efficient methotrexate treatment. 
INTRODUCTION 
With the expression "actinic reticuloid" (AR) Ive et al (1) 
described 10 elderly male patients who developed chronic 
photosensitivity which, whenchanges were severe. suggested 
lymphoma. The eruption begins asa slightly scaly erythema 
which extends rapidly over the whole head. neck and backs 
of hands, often also to covered areas. In the established 
disease there is little scaling and the erythema is accompanied 
by edematous thickened plaques and smooth topped papules. 
Erythrodermic episodes were seen in most patients and were 
not related to seasonal changes. The lack of recognition of 
photosensitivity by the patients and even by the doctors was 
acta dermatovenerologica A.P.A. \lo/ l, 92, No./ 
due to the absence of seasonal fluctuations and to the presence 
of lesions on the covered parts of the skin. Confinement to a 
dark room produced improvement in almost all patients. 
Histopathology usually reveals a cellular infiltrate in the 
papillary de1mis composed of 1 ymphocytes and histiocytes, 
but some eosinophils, plasma cells. giant cells and a small 
number of atypical mononuclear cells can be found. The 
occasional epidermal invasion may simulate lymphoma. 
Toonstraet al (2) described the presence oflymphocytes with 
convoluted nuclei, blast cells, multinucleated fibroblasts and 
mitotic figures. Immunophenotyping showed a predominance 
of suppressor T cells (2). 
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Acti11ic reticuloid-like CTCL 
Ramsay (3) believes thata photosensitization is responsible 
for the development of AR, halogenated salicylanilides as 
tetrachlorsalicylanilide and hexachlorophen which are often 
added as antimicrobial agents to soaps and cosmetics might 
be the cause. The expression persistent light reactor coined 
by Wilkinson is also mentioned in this context (4). In such 
patients ahypersensitivity to light persists for years, while the 
provoking chemicals remain undetected. The authors who 
stick to such a definition believe that AR does not progress to 
definite malignant lymphoma (3). 
On the other hand, the "reticuloid" in the name of this 
disease or rather syndrome suggests that the pathohistology 
resembled what we used to call reticuloses and today designate 
as malignant lymphomas (5) . Certain authors associate AR 
with Sezary syndrome the main clinical features of which 
are: erythroderma with infiltration and scaling of the skin, the 
face gives often the impression of so called facies leonina, 
pruritus, peripheral lymphadenopathy, diffuse palmoplantar 
hyperkeratosis, subungual hyperkeratosis and the presence 
of cells with cerebriform nuclei (Lutzner cells) in the skin 
and peripheral blood. Two groups of patients can be 
distinguished within the Sezary syndrome: 
1. patients with erythrodermic cutaneous T cell lymphoma 
and, 
2. patients with AR or chronic actinic dermatitis (6). In the 
first group the circulating Sezary cells were characterized as 
predominantly OKT 4 positive T cells (helpers), while in the 
second group they were predominantly OKT 8 positive 
(suppressors). 
The fact that one of the original 10 AR patients observed 
by Ive ( 1) developedleukemiaspeaks infavorof the hypothesis 
that AR may evolve in the direction of malignant lymphoma. 
This short introduction shows that certain questions 
conceming AR and Sezary syndrome still remain unanswered. 
CASEREPORT 
The 52-year-old patient enjoyed a good health except for 
a number of injuries and fractures. In summer 1992 itching 
appeared on the palms and soles which soon extended to the 
entire surface of the feet and hands. To ihe diseased areas of 
the skin he applied ointments obtained from his general 
practitioner. Later on. his skin condition worsened so that the 
whole body became involved, which the patient associated 
with spraying of pesticide s in his vineyard. 
In August he was admitted to the Department of 
dermatology of the General Hospital in Maribor. At that tirne 
an almost complete erythroderma was expressed with an 
120 
enlargement of the axillary and inguinal lymph nodes. A 
biopsy of the involved skin was read as mycosis fungoides 
(MF). 
On october 6, 1992, he was transferred to the Institute of 
Oncology in Ljubljana. At that tirne the whole skin was red 
with scaling and partially infiltrated. On palms and soles 
there was a marked hyperkeratosis with rhagadae and the 
nails were thickened. In the jugular region a non sharply 
demarcated plaque with a diameter of approximately 2 cm 
was expressed, while two similar lesions were present in the 
left anterior axillary fold as well as above the left os ilii. In 
both axillae fused lymph nodes approximately 4 cm in 
diameter were palpable. Inguinal and femoral lymph nodes 
were also enlarged and fused. Lymph nodes on the neck were 
palpable too. The !iver and the spleen were not enlarged. The 
routine laboratory tests were within normal limits, except for 
a slight leucocytosis in the peripheral blood. The X ray of the 
chest wasnormal. The ultrasound investigation of the abdomen 
disclosed two hyperechogenic zones in the right !iver lobe 
which were interpreted as hemangiomas. Fine needle 
aspiration biopsies of the axillary and inguinal lymph nodes 
as well as of the skin revealed a non-Hodgkin lymphoma. 
Histopathology of a biopsized axillary 1 ymph node showed a 
non-Hodgkin 1 ymphoma of a high degree of malignancy: T-
cell type, pleomorphic of medium and large cells CD 3 +, 
MTl +/-, UGHL 1 -, OPD4-/+, L 26-,MB 2-,BerH 2-. An 
increased number of venules with high endothelial cells, also 
apositiveimmunologicreactiontofactorVIIIandarelatively 
Fig . 1. Actinic reticuloid-like cutaneous T celi lymphoma 
(CTCL). Erythema and hyperkeratosis with rhagadae 011 the 
pa/ms. Thickned nails. 
Fig. 2. Actinic reticuloid-like CTCL. Simi/ar changes on the 
soles. 
Fig. 3. Actinic reticuloid-like CTCL. The skin oj thejace is 
edematous, reddened and i11filtrated. 
Fig. 4. Pacal lymphoid injiltrates in the dermis are shown 
with a discretejocus oj epidermotropism. H and E. 
Fig. 5. Higher power view oj the lymphoid i11filtrate within 
the dermis. Most oj the lymphoid cells are oj the medi um size 
with some nuclear convolutions. Larger cells with more 
prominent nucleoli are also seen. H and E. 
Fig. 6. Lymph node with diffuse i11filtration by lymphoma 
cel/s oj medi um siže with convofuted nuclei and individual 
large cel/s with larger nucleoli. An atypical mitoticjigure is 
also seen. H and E. 
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Actinic reticu/oid-like CTCL 
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Acrinic reticuloid-like CTCL 
increased number of macrophages/histiocytes (CD 68 
positive) confirmed the above mentioned diagnosis. In the 
skin, focal perivascular lymphoid infiltrates with the same 
cytological population was found with focal discrete 
epidermotropism. Histopathology and immunotypisation 
were interpreted as that of a primary nodal lymphoma with 
secondary penetration into the skin. The absence of Pautrier's 
microabscesses excluded MF. 
Further assaying of peripheral blood lymphocytes disclosed 
that 75 % were T lymphocytes and only 1 % were B, out of 
the T 54 % were CD 4 and 43 % CD 8 giving a CD 4/CD 8 
ratio of 1.27. Activity ofNK cells was 13 %, the lymphocyte 
transformation test using PHA and Con A was within normal 
limits. No Sezary cells were seen. 
Treatment may be shottly summarized as follows. Oi 
October 20, 1992 a chemotherapy with Cyclophosphamid. 
Adrioblastin, Vincristin and Bleomycin was introduced 
according to the CHOP-Bleo scheme. Later on futther 
chemotherapeutics like Endoxan and Oncovin were included 
into the treatment. After almost three months only amoderate 
improvement was noted, with persisting erythrodem1a and 
the lymph nodes stili enlarged. For this reason the previous 
chemotherapeutic scheme was substituted by 180 mg metho-
trexate applied in infusion once weekly and prednisolon 40 
mg daily. This treatment proved to be far more efficient. On 
February 4 there was neither erythrodermanor skin infiltration 
present. the skin was however slightly brownish pigmented. 
The diffuse hyperkeratoses on the palms and so les as well the 
rhagadae disappeared, on the soles persisted small areas of 
hyperkeratosis. It is important to note that the skin ofthe face 
remained reddened and on the neck it was stili somewhat 
infiltrated, thus giving the impression of persisting 
photosensi ti vi ty. 
DISCUSSION 
The skin lesions in our patient were almost identical with 
the T-cell lymphoma described as Sezary syndrome: 
erythroderma with scaling. edema and infiltration of the 
facial skin giving the impression of the so called facies 
leontina, diffuse hyperkeratosis of palms and soles with 
rhagadae. subungual hyperkeratosis with defonnation ofthe 
acta dermaro\'enerologica A.P.A. Vol 1, 92, No 4 
nails, a diffuse alopecia and an enlargement of the peripheral 
lymph nodes (5). In the affected skin as well as in the lymph 
nodes there were small and large cells with relatively large 
convoluted nuclei and scarce cytoplasma, although typical 
cerebriform nuclei were not detected. 
Studies of the function of the pathological lymphocytes 
have indicated that in the Sezary syndrome they have the 
functional capacity to help other lymphocytes in theirrole of 
immunoglobulin production and were identified as bearirig 
the membrane markers of T helper cells, OKT 4 positive (7). 
During the last few years a number of papers on 
immunophenotypes of the neoplastic celi s in T-cell I ymphoma 
appeared (8). Patients in which the CD 8 suppressor/cytotoxic 
T cells prevailed had an advanced or fulminant disease, so 
that this phenotype seems to be associated with a poor 
prognosis (9). Even within these patients two groups may be 
discemed: one more rapidly progressing (CD 2-, CD 7+) and 
anotherrather chronic (CD 2 +. CD 7 -). On the otherhand T 
Cell lymphomas with CD 4 + seem to have even in persons 
under 30 years of age a relatively good prognosis. 
According to the data which can be obtained in the textbooks 
actinic reticuloid, mycosis fungoides, Sezary syndrome and 
CTCL of a high degree of malignancy are clearly defined 
nosologic entities. Clinicians however sometimes encounter 
cases which are hard to assign to one of these entities. In the 
literature are even mentioned cases of Sezary syndrome 
without skin manifestations ( 11 ). 
The diagnosis of actinic reticuloid-like lymphoma 
cutaneous T cell lymphoma characterizes best our patient's 
condition for the following reasons: both the pathologist and 
the cytologist interpreted definitely the bioptic material as a 
non-Hodgkin lymphoma of high degree of malignancy, the 
clinical manifestations resembled the Sezary syndrome; there 
were no Lutzner cells in the peripheral blood and there was 
expressed a photosensitivity of a milder degree. 
We believe that ourpatient posesan interesting diagnostical 
and therapeutical problem. It can be assumed safely that by 
introducing more sophisticated laboratory methods as 
genomic DNA analysis, studies of cytokine secretion patterns 
as well as other methods it will be easier to solve such 
problems (12, 13. 14). 
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Actinic reticuloid-like CTCL 
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13. Vowels BR, Cassin M, Vonderheid EC et al. Aben-ant 
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Invest Dermatol 1992; 99:90-94. 
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Sezary syndrome. J Amer Acad Dermatol 1991; 25:731-2. 
AUTHORS' ADDRESSES 
124 
Petrič -Grabnar Gabrijela M.D., oncologist, Institute of Oncology. Zaloška 4, 61000 Ljubljana, Slovenia 
Lamovec Janez M.D., Ph.D., pathologist, same address 
Aleksej Kansky M.D., Ph.D„ professor of de1matology, Štihova 26, 61000 Ljubljana, Slovenia 
acta dermatovenerologica A.P.A. Vol 1, 92 , No 4