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Annular erythema nodosum leprosum reaction in a
young male: a rare presentation
The presentation of erythema nodosum leprosum lesions in an
annular pattern is rare.
Herein, we report a case of lepromatous leprosy (LL) in a
farmer who presented with erythema nodosum leprosum lesions
arranged in an annular pattern.
A 45-year-old farmer presented with painful nodules on the
face and back of 1-week duration, associated with fever, joint
pains, and malaise. He had been diagnosed with LL and com-
pleted World Health Organization multibacillary, multidrug ther-
apy the previous year. There was a history of similar nodules
three months prior to his diagnosis of LL for which he was man-
aged with a short course of systemic steroids. Family history for
leprosy was negative. Examination revealed multiple faintly ery-
thematous nodules distributed on the face, back of the trunk,
and legs; nodules were tender and soft to ﬁrm in consistency.
The nodules on the face and back of the trunk were distributed
in an annular pattern bilaterally (Fig. 1). Glove and stocking
anesthesia was evident. Both greater auricular nerves, left
ulnar, right lateral popliteal, and left posterior tibial nerve were
thickened and nontender. He was diagnosed as lepromatous
leprosy with type 2 reaction. All the hematological and biochem-
ical investigations were unremarkable. Venereal disease
research laboratory (VDRL) test and human immune deﬁciency
virus (HIV) serology 1 and 2 were nonreactive. Slit skin smear
from nodule and ear lobules revealed granular Acid-fast bacilli
(Fig. 2a,b). Histopathological examination of biopsy, which had
been taken from a representative nodule, showed periadnexal
moderate inﬁltrate of lymphocytes, histiocytes, foam cells, and
few neutrophils in the dermis. Subcutaneous fat showed lobular
panniculitis (Fig. 2c,d).
The term erythema nodosum leprosum (ENL) was ﬁrst coined
It has been reported that 15–50% of lepromatous
leprosy patients develop ENL in the ﬁrst year of treatment,
its severity is related to the high bacterial index.
encountered mainly in lepromatous leprosy and occasionally in
borderline lepromatous leprosy. Precipitating factors include
intercurrent infection, pregnancy, surgery, vaccination, and phy-
sical and mental stress.
It is characterized by evanescent, ery-
thematous, tender nodules distributed mainly on the front of the
chest, arms, thighs, and face; it is usually associated with
constitutional symptoms like fever and arthralgia. It can involve
various organs and manifest as lymphadenitis, iridocyclitis,
epididymo-orchitis, glomerulonephritis, synovitis, dactylitis, and
Immunologically, ENL is an Arthus reaction, characterized
by vasculitis as a result of deposition of immune complexes
and complement around blood vessels with inﬁltration of poly-
morphonuclear leukocytes. It has long been regarded as
basic pathology for the development of ENL; however, of late,
cell-mediated immunity (CMI) is also considered important in
the causation of ENL. Kahawita and Lockwood carried out a
critical analysis of ENL lesions and found that the major
T-cell subtype in ENL was the CD4 cell in contrast with
lepromatous leprosy where CD8 cells predominate. Moreover,
the cytokines, TNFa, and IL-6 were consistently found whilst
IL-4 was low or absent in ENL, indicating a Th1 type
response. Additionally, keratinocyte 1a and intercellular adhe-
sion molecule-1 (ICAM-1) were also found in the epidermis in
ENL, which is also indicative of a cell-mediated immune
The distribution of ENL lesions in circinate fashion to form
two annular plaques, one on either side of the back of the trunk,
in the index case appears to be novel. Notably, these plaques
are distributed bilaterally and symmetrically with grouping in the
left plaque. Moreover, the ENL lesions on the left cheek also
(Original) (a) Multiple ENL lesions arranged in
annular plaques on either side of the back. (b) Annular
plaque of ENL on left malar region. (c) ENL lesions showing
a tendency to form plaque on the right cheek
International Journal of Dermatology 2018, 57, 490–501 ª 2018 The International Society of Dermatology