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Fine needle aspiration cytologic diagnosis of erythema nodosum leprosum: A

case report

Article  in  Acta Cytologica · September 2007

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CASE REPORTS

Fine Needle Aspiration Cytologic Diagnosis of

Erythema Nodosum Leprosum
A Case Report

TE
Neelaiah Siddaraju, M.D., Sumit Kumar Roy, M.D.,
Manish Mahadeorao Bundele, M.D., Bhavana Ashok Badhe, M.D., and
Devinder Mohan Thappa, M.D.

A
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Background Conclusion
Erythema nodosum leprosum (ENL), the type 2 lepra reac- Cytologic features such as a large number of intact and de-

I
tion occurring in lepromatous or borderline lepromatous generated neutrophils with foamy macrophages and strong
leprosy, presents clinically with granular AFB positivity, in

L
acute manifestations that an appropriate clinical back-
compel the patient to seek Our case emphasizes the ground, allows a confident di-
medical attention. Recognition importance of clinical findings and agnosis of ENL. (Acta Cytol
P
l
ria
and timely management of 2007;51:800–802)
these patients is critical in the role of simple FNA technique in

te
U
order to avoid permanent dis- the cytodiagnosis of ENL. Keywords: aspiration cy-
a tology, fine-needle; erythe-
M
ability. Fine needle aspiration
cytology (FNAC) is a simple, ma nodosum leprosum;
D
ed

effective tool that aids in correct diagnosis and management leprosy.

of ENL.
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L
eprosy is a slowly progressive infection caused by
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Case Mycobacterium leprae. It affects chiefly the skin and

T
y

A 30-year-old woman presented with history of fever, red- peripheral nerves, resulting in disabling deformities.1
op

dening of the face, and multiple raised, reddish, painful The Ridley-Jopling system classifies leprosy into 5
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swellings of the bilateral forearms and legs for 7 days. One clinically and histologically recognizable categories.
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year previously, she was diagnosed and treated for leproma- Tuberculoid leprosy and lepromatous leprosy form
©

tous leprosy with type 2 reaction. After a thorough clinical the 2 poles, and the middle of the spectrum consists of
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examination a diagnosis of ENL was made. FNA smears
from the forearm swellings showed pus-like material with
intact and degenerated polymorphonuclear leukocytes and
many foamy macrophages with strong granular acid-fast
O
borderline tuberculoid (BT), mid-borderline or bor-
derline borderline (BB) and borderline lepromatous
(BL) categories.2 Leprosy patients may develop 2
types of reactions. The type 1 reaction is the reflection

bacillus (AFB) positivity. A cytologic diagnosis of ENL was of immunologic instability accompanying the disease
given, which was confirmed on histopathologic examination that occurs in borderline leprosy of BT, BB and BL
of skin biopsy. types; the type 2 reaction, erythema nodosum lepro-
D

From the Departments of Pathology and Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry,
India.
Drs. Siddaraju and Badhe are Professors, Department of Pathology.
Drs. Roy and Bundele are Senior Residents, Department of Pathology.
Dr. Thappa is Professor and Head, Department of Dermatology.
Address correspondence to: Neelaiah Siddaraju, M.D., Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and
Research, Dhanwantari Nagar, Ponicherry, 605 006 India (rajusiddaraju@yahoo.com).
Financial Disclosure: The authors have no connection to any companies or products mentioned in this article.
Received for publication February 28, 2006.
Accepted for publication April 19, 2006.

800 ACTA CYTOLOGICA 0001-5547/07/5105-0800/$19.00/0 © The International Academy of Cytology


sum (ENL), is a reflection of immune complex phe-
nomena of the Arthus type that occurs in lepromatous
and borderline lepromatous cases. These reactions are
responsible for acute clinical symptoms that occur in
the otherwise indolent course of leprosy.3 Recogni-
tion of the reactionary state and a prompt initiation of
therapy are highly essential to prevent permanent dis-
ability that occurs as a result of the accelerated nerve
damage or the damage of other organs occurring dur-
ing an episode.4 We report a case of ENL diagnosed
on fine needle aspiration cytology (FNAC) to high-
light the clinical utility of the simple FNA technique
in reactionary leprosy.
Case Report
A 30-year-old woman was admitted in the skin ward
with history of fever, reddening of the face, and mul-
tiple raised, reddish, painful swellings of the bilateral
forearms and legs for 7 days. On examination, multi-
ple, discrete, tender erythematous nodules and
papules were seen over the ventral as well as dorsal as-
pect of the bilateral forearms and legs. Bilateral ulnar,
radial and common peroneal nerves were thickened
and tender. A decreased sensation over the medial as-
pect of the dorsum of the left foot was noted. There
were no deformities. It was also found that a year pre-
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viously she had similar lesions with clinical and histo-
logic investigations, revealing the presence of lepro-
M
matous leprosy with a type 2 reaction. At that time,
D
ed
she received anti-leprosy treatment, along with pred-
nisolone, but was lost to follow-up after 4 months of
treatment. Based on these features, a clinical diagnosis
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of erythema nodosum leprosum (ENL) was made.
T
y
Laboratory investigation revealed anemia with a he-
op
moglobin of 7.3 g/dL, neutrophilic leukocytosis and
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C
©
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O
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Figure 1 Cytologic smear showing intact and degenerated
polymorphs and macrophages in a necrotic background
(MGG stain, × 200). Inset highlights intracellular granular acid-fast
bacilli (Fites stain, × 1,000).
Volume 51 Number 5
FNAC in Erythema Nodosum Leprosum
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A
Figure 2 Histologic section of erythema nodosum leprosum
(hematoxylin-eosin, × 200).
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left shift of neutrophils, with a total leukocyte count of
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31.2 × 109/L and a platelet count of 481 × 109/L. ESR
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was 80 mm at the end of the first hour. Split skin smear
from the ear lobes showed acid-fast bacilli ++++ (AFB
4+). FNA performed on the forearm swellings, using a
P
l
ria
23-gauge needle, attached to a 10-mL disposable sy-
ringe, yielded pus-like material. Air-dried May-
te
Grünwald-Giemsa (MGG) and alcohol-fixed Papani-
a
colaou smears were studied. The smears showed a
large number of intact and degenerated polymor-
phonuclear leukocytes and several scattered foamy
macrophages in a necrotic background, along with an
ht
occasional cluster of endothelial cells. In view of the
clinical findings and diagnosis, Fites staining for AFB
was performed, which showed strong granular positiv-
ity (Figure 1). Cytologic diagnosis was consistent with
the clinical diagnosis of ENL, which was confirmed
subsequently by the histopathologic examination of
skin lesion biopsies from the forearm swelling (Figure
2).
Discussion
In the past, histopathologic examination was the only
way of confirming a clinical diagnosis of leprosy. More
recently, the role of cytology in its diagnosis has been
emphasized by various authors.5,6 Cytology in the
form of slit skin smears has been in regular use for as-
sessing leprotic lesions.6 Later studies have attempted
with fair success to diagnose and categorize leprosy,
according to the Ridley-Jopling classification, based
on cytologic findings of cytopuncture, slit skin smear
and FNA techniques.6 Other authors have empha-
sized the role of FNAC in detection of the nerve in-
volvement in leprosy.7,8 As for the cytodiagnosis of re-
actionary leprosy, there have been rare studies.5,9
Singh et al5 encountered 4 cases of ENL in their series
September–October 2007 ACTA CYTOLOGICA 801
 Siddaraju et al
of 22 cases of lepromatous leprosy, and Malik et al9 di-
agnosed 7 cases of ENL in their series of 42 cases of
clinically diagnosed reactionary leprosy. In their
study, Malik et al described the cytomorphologic fea-
tures of types 1 and 2 reactions and stated that
histopathologic criteria used in the diagnosis of reac-
tionary leprosy can be satisfactorily applied to cyto-
logic smears.9 As for more recent studies, there has
been only a single case report of erythema nodosum
leprosum diagnosed on FNAC.10
Clinically, ENL occurs in 25–75% cases of lepro-
matous leprosy11 and occasionally in borderline lepro-
matous forms.12 It develops most frequently in the lat-
ter half of the initial year of treatment. The lesions
consist of crops of tender, inflamed, subcutaneous
nodules that generally last for 1 or 2 weeks. Low-
grade fever, generalized lymphadenopathy,13 nerve
involvement10 and arthralgias13 can accompany severe
ENL. Histologically, ENL shows an intense neu-
trophilic infiltrate within a lepromatous macrophage
granuloma containing a large number of fragmented
and granular bacilli, along with swelling and edema of
the endothelium and neutrophilic infiltration of vessel
walls.10,14
Consistent cytologic features described in the cases
of ENL reported so far include a large number of neu-
U
trophils, foamy macrophages and strong AFB positiv-
ity.5,9,10 Other features, not so consistently reported,
M
are high cellular yield, lymphocytes, blood vessels,
D
ed
collagen and elastin and fatty background.9 Anshu et
al10 observed intracellular and extracellular bacilli in
the form of negative images on MGG-stained smears;
Singh et al5 state that negative images are unusual in
T
y
ENL because bacilli are frequently fragmented. In our
op
case, FNA yielded pus-like material that showed a
O
large number of intact and degenerated neutrophils,
C
along with some foamy macrophages and an occasion-
©
al group of endothelial cells. Generally, these cytolog-
N
ic features suggest a suppurative lesion, but in the
present case, in view of the clinical findings and diag-
nosis, Fites stain for AFB was performed, which
demonstrated granular bacilli. However, no intracel-
O
lular or extracellular negative images of bacilli were
observed on MGG-stained smears. With these fea-
tures, we could render a definitive cytologic diagnosis
D
of ENL, which was confirmed by histopathologic
examination.
802 ACTA CYTOLOGICA Volume 51 Number 5 September–October 2007
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Our case emphasizes the importance of clinical
findings and the role of simple FNA technique in the
cytodiagnosis of ENL. Cytologic features such as a
large number of polymorphs with foamy macrophages
and strong granular AFB positivity, in an appropriate
clinical background allows a confident diagnosis of
ENL and helps avoid unnecessary skin biopsy.
References
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