EXTRAORDINARY CASE REPORT
Mycosis Fungoides Manifesting as Giant Cell Lichenoid
Dermatitis
Irena E. Belousova, MD, PhD,* Vladislav R. Khairutdinov, MD, PhD,* Anna Bessalova, MD, PhD,†
and Dmitry V. Kazakov, MD, PhD‡
Abstract: Mycosis fungoides (MF) is the most common form of
primary cutaneous lymphoma with a broad clinicopathological
spectrum. Unusual histopathologic patterns of MF include lichenoid,
interstitial, folliculotropic, spongiotic, granulomatous, and many
others. Several cases of unusual lichenoid reaction characterized
by a mixed lichenoid inflammatory infiltrate with prominent
infiltration of the papillary dermis and epidermis by multinucleated
giant cells were described under the name of “giant cell lichenoid
dermatitis,” most of them were considered to represent a drug erup-
tion. Herein, we describe a 77-year-old woman with a 5-year history
of MF displaying microscopic features of giant cell lichenoid der-
matitis. Histology revealed a dense band-like lichenoid epidermo-
tropic infiltrate composed of CD4+ small to medium-sized
lymphocytes with cerebriform nuclei with the presence of multinu-
cleated giant cells in the papillary dermis, within the epidermis, and
some hair follicles. Monoclonal TCR gene rearrangement was de-
tected using PCR. To the best of our knowledge, this pattern was
never described in MF.
Key Words: mycosis fungoides, giant cell lichenoid dermatitis,
granulomatous mycosis fungoides, granulomatous slack skin
(Am J Dermatopathol 2017;0:1–3)
M ycosis fungoides (MF) is the most common form of primary
cutaneous lymphoma with a broad clinicopathological spec-
trum.1–4 Apart from the classic microscopic presentation with epi-
dermotropism of small lymphocytes, unusual histopathologic
patterns of MF include lichenoid, interstitial, folliculotropic, spongi-
otic, granulomatous, and many others.3 In 1968, an unusual lichen-
oid reaction characterized by a mixed lichenoid inflammatory
infiltrate with prominent infiltration of the papillary dermis and epi-
dermis by multinucleated giant cells was described under the name
of “giant cell lichenoid dermatitis” (GLD). It was believed to repre-
sent a drug eruption in a patient with a history of systemic lupus
erythematosus that resolved after discontinuation of the medica-
tions.5 To date, only few cases of GLD have been reported in the
literature.6–9 Most of them were considered to represent a drug erup-
From the *Department of Dermatology, Medical Military Academy, Saint
Petersburg, Russia; †Department of Pathology, North-Western State Med-
ical University, Saint Petersburg, Russia; and ‡Sikl’s Department of Pathol-
ogy, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech
Republic.
The authors declare no conflicts of interest.
Reprints: Dmitry V. Kazakov, MD, PhD, Sikl’s Department of Pathology,
Charles University Medical Faculty Hospital, Alej Svobody 80, 304 60
Pilsen, Czech Republic (e-mail: kazakov@medima.cz).
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tion,5,6,9 whereas other associations included sarcoidosis6 and li-
chenoid eruptions within herpes zoster scars in a patient with an
acute lymphoblastic leukemia8 or believed to represent an idiopathic
reaction.10 Most recently, a case of granulomatous lichenoid MF has
been published where the authors described a similar histological
pattern but without multinucleated giant cells in the epidermis.11
Herein, we report a case of MF displaying microscopic features of
GLD, including intraepidermal giant cells, a pattern not previously
described in this lymphoma, to the best of our knowledge.
CASE REPORT
The patient was a 77-year-old woman with a 5-year history of
slowly progressive erythematosquamous patches and plaques. Ac-
cording to the patient and her medical documentation, the disease
started as a single erythematous patch on her back and slowly
enlarged. Subsequently, new multiple lesions developed on the body
and extremities that slowly progressed and became itchy. Various
clinical diagnoses were rendered, including allergic dermatitis, drug
eruption, eczema, and psoriasis. Treatment with topical cortico-
steroids resulted in partial regression but soon the lesions became
confluent and widespread with prominent scaling and itching (Figs.
1A, B). Palmar and plantar hyperkeratosis developed (Fig. 1C). The
patient’s medical history included hypertension, coronary artery dis-
ease, Parkinson disease, nephrolithiasis, and chronic pyelonephritis.
The patient denied regular intake of any medications apart from
antihypertensive drugs. In addition to the skin lesions, physical
examination revealed periphery lymphadenopathy. Peripheral blood
tests revealed relative lymphocytosis (52%), whereas the total
lymphocyte count was normal. Computed tomography of the chest
and abdomen disclosed multiple enlarged axillary lymph nodes and
a conglomerate of enlarged lymph nodes in the anterior
mediastinum.
Two skin biopsies were obtained (time span between biopsies
was 3 months), both of which revealed identical changes. There was
an acanthotic epidermis with hyperkeratosis and a dense band–like
lichenoid epidermotropic infiltrate composed of small to medium-
sized lymphocytes with cerebriform nuclei admixed with plasma
cells and histiocytes. Focally, lymphocytes formed small microab-
scesses within the epidermis and follicular infundibula. A conspicu-
ous feature was the presence of multinucleated giant cells in the
papillary dermis, within the epidermis, and some hair follicles. The
cytoplasm of these cells was either empty or contained powdery
melanin (positive on Masson-Fontana stains). In addition, emperi-
polesis of lymphoid cells was seen. Focally, intraepidermal giant
cells were surrounded by aggregates of small cerebriform lympho-
cytes (Figs. 2 and 3).
Immunohistochemically, most lymphocytes were positive for
CD3, CD4, and CD5, with an increased CD4:CD8 ratio (approxi-
mately 5:1) (Fig. 2D). Rare CD30+ cells were scattered in the infil-
trate. Small clusters of CD20+ B-lymphocytes were evident at the
periphery of the infiltrate. Monoclonal TCR gene rearrangement was
detected using PCR in both specimens, with clones of identical
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Belousova et al Am J Dermatopathol  Volume 0, Number 0, Month 2017

FIGURE 1. Confluent and widespread

erythematosquamous patches and
plaques with prominent scaling almost
amounting to erythroderma (A and B)
and palmar and plantar hyperkeratosis
(C).

length. A lymph node biopsy revealed features of dermatopathic
lymphadenopathy.
The patient was treated with UVB, which resulted in rapid
partial regression of the lesions. The treatment was discontinued
because of severe itching, and methotrexate 30 mg and interferon
alfa 9 MU/mL weekly have been administered with good partial
response.
DISCUSSION
Albeit drug eruption was a diagnostic consideration in
our patient (due to the previously published material, where
most cases of GLD were associated with drug intake), the
diagnosis of MF was based on the history of progressive
disease, epidermotropic infiltrates of small cerebriform lym-
phocytes, clonal rearrangement of TCR genes with identical
clones in 2 separate biopsies, and good response to therapy.
As far as we are aware, GLD with prominent intraepidermal
giant cells has never been reported as a microscopic mani-
festation of MF. Garrido et al has recently published a very
similar occurrence; however, intraepidermal/intrafollicular
multinucleated giant cells were lacking in their case.11
In the original article describing GLD, marked exo-
cytosis of lymphoid cells in the follicular epithelium with
minimum spongiosis is evident, a feature prompting a con-
sideration of MF (Fig 4 in 5). The patient showed partial

FIGURE 2. A dense band–like

lichenoid epidermotropic infiltrate
composed mainly of small lympho-
cytes with cerebriform nuclei (A). In-
trafollicular multinucleated giant cells
surrounded by small cerebriform
lymphocytes (B). Multinucleated
giant cells both within the dermis and
the epidermis containing powdery
melanin. Note emperipolesis of lym-
phoid cells (C). Staining for CD4. Note
epidermotropism of lymphocytes and
CD4-positive multinucleated cells
within the epidermis (D).

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Am J Dermatopathol  Volume 0, Number 0, Month 2017
FIGURE 3. Close up: epidermotropism of small to medium-
sized lymphocytes.
remission during the next 10 weeks, but the long-term follow-
up is unknown. Histological specimens of 2 more patients
reported with GLD and who were considered to have drug
eruption also demonstrated mild spongiosis with exocytosis
of lymphocytes6; one of them developed hyper- and hypo-
pigmented lesions that never resolved completely, whereas
the lesions in another patient resolved.
One of the reported patients with GLD was also
diagnosed with pulmonary sarcoidosis.6 This is likely an inci-
dental feature, as the classic morphology of cutaneous sar-
coidosis is characterized by noncaseating epithelioid
granulomas lacking a peripheral cuff of lymphocytes. Our
patient had enlarged mediastinal lymph nodes and peripheral
lymphadenopathy. Although the nature of mediastinal lymph-
adenopathy is unclear, the axillary lymph node biopsy re-
vealed features of dermatopathic lymphadenopathy, which
is seen in peripheral T-cell lymphoma, especially in Sezary
syndrome or erythrodermic MF. In our patient, cutaneous
involvement at the time of lymph node biopsy almost
amounted to eythroderma, and marked lymphocytosis was
detected; however, as no Sezary cells were found in the
peripheral blood, the diagnosis of Sezary syndrome was not
rendered.
Giant cells with emperipolesis are a typical feature of
granulomatous slack skin (GSS), a condition related to MF.
GSS is characterized clinically by the occurrence of slowly
developing, bulky, infiltrated folds of atrophic skin in flexural
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Mycosis Fungoides
areas, mainly in the groin and axilla. GSS manifests
histopathologically by a dense epidermotropic infiltrate
composed of small convoluted lymphocytes accompanied
by numerous multinucleated giant cells containing 20–30
nuclei arranged in a wreath-like fashion that engulf the lym-
phoid cells (emperipolesis) and contain degenerated elastic
fibers in their cytoplasm. In contrast to GLD in our case
where the lichenoid infiltrate was confined to the upper der-
mis, the infiltrate in GSS usually involves the whole dermis,
where elastic fibers are almost totally absent.
GLD is a very rare condition that has a distinctive
histopathological pattern. However, some authors have used
the term GLD for a somewhat different picture.7 Magro et al
reported 40 patients with a band-like lymphocytic infiltrate
with concomitant granulomatous inflammation but as far as it
can be judged from the illustrations and description, intra-
epidermal giant cells were lacking.
In summary, we have described a patient with MF
whose histological picture matched the original description of
GLD, with a lichenoid infiltrate and multinucleated cells
within the epidermis, hair follicle epithelium, and papillary
dermis. This case extends the associations of GLD, most of
which seem to be a manifestation of a drug reaction.
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