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Histoid
leprosy is a rare clinical variant of leprosy. Several cases have been reported
from India even in post-elimination era. Histoid leprosy mimics variety of
dermatological conditions like xanthoma, neurofibroma, dermatofibroma, sarcoid
and post-kala-azar leishmaniasis. We report two cases of histoid leprosy
mimicking eruptive xanthomas in different age groups.
Keywords: Histoid leprosy, Clinical features, Xanthomas, Skin smears
INTRODUCTION
Histoid leprosy
(HL), a variant of lepromatous leprosy (LL), was first described by Wade in the
year 1960 [1]. Large number of cases has been reported from India and other
endemic countries, even in post elimination period [2,3]. In India its
incidence among patients with leprosy is estimated to be 2.79%-3.60% [4].
CASE 1
A 65 year old
non-diabetic male of Indian origin, consulted a dermatologist in New Jersey,
USA for asymptomatic, multiple, skin colored, shiny, nodules over the back,
trunk and extremities. He was thoroughly investigated. All the hematological
investigations were normal. The biochemical tests were: fasting blood sugar:
104 mg/dl; HbA1c: 7%; lipid profile (cholesterol: 230 mg/dl; triglycerides: 300
mg/dl; HDL: 35 mg/dl; LDL: 145 mg/dl). Other biochemical tests like liver
function tests, blood urea, serum creatinine were within normal limits. Skin
biopsy report showed a collection of CD68 positive foam cells in upper part of
dermis. CD117 highlights rare mast cells. Immunohistochemical (IHC) markers
showed scattered Langerhans cells expressing S-100. Skin smear examination was
not done. A diagnosis of xanthoma was made. He was advised regarding diet and
regular exercise. After 6 months, he came to his native place in India and
consulted three more local dermatologists as his condition worsened. They were
also of the same opinion basing upon the USA reports and he was not advised any
specific treatment. As his clinical condition was worsening with development of
newer lesions, he visited our department for opinion. On our examination, we
found multiple, discrete, skin colored, shiny, succulent, nodular lesions and
plaques all over the back, trunk, and both upper and lower extremities on
apparently normal skin (Figure 1). A
few nodules were also seen on the face and both ear lobules. Edema of both
hands and feet with non-healing ulcers over the fingers were also present. Both
ulnar and lateral popliteal nerves were thickened and sensory impairment over
the lesions and glove and stocking anesthesia were present. There was no
lymphadenopathy and organomegaly. Our clinical diagnosis was histoid leprosy or
lepromatous leprosy. In addition to routine hematological and biochemical
investigations, skin smears for acid fast bacilli (AFB) were taken from ear
lobes as well as from nodules and plaques and skin biopsy was taken from the nodules.
Skin smears showed multiple solid staining AFB, no bundles and globi were
observed (Figure 2). Bacterial index
was 6+. Lesional skin biopsy specimen revealed the following features,
epidermis was thinned out and multiple granulomas comprising histiocytes,
lymphocytes, plasma cells and foam cells were present in the dermis. Occasional
spindle shaped histiocytes were also present (Figure 3a). Fite stain of the specimen showed solid AFB (Figure 3b). A final diagnosis of
histoid leprosy was arrived on the basis of clinical, histopathological and
bacteriological features. He was advised multibacillary-multidrug therapy
(MB-MDT) WHO regimen comprising dapsone 100 mg and clofazamine 50 mg daily with
rifampicin 600 mg and clofazamine 300 mg once in a month under supervision for
2 years. In view of high bacterial load, ofloxacin 400 mg twice daily for 8
week
CASE 2
An 8 year old
male child with multiple asymptomatic skin colored nodules was referred from
the department of pediatrics with a diagnosis of xanthoma for our opinion. On
physical examination, there were multiple, discrete, asymptomatic skin colored
nodules over inner thighs, scrotum and penis. In addition, there were
infiltrated plaques over buttocks (Figure
4). Detailed history revealed the child started developing these lesions
initially on the thighs 6months ago and multiple lesions over the other areas
during the course of illness. Sensory impairment over the lesions and lower
extremities was equivocal. Bilateral popliteal nerves were thickened and
non-tender. There was no regional lymphadenopathy. Systemic examination was
normal. All hematological and biochemical investigations were normal. Skin
smears from the nodules were positive for AFB and bacterial index 6+. Lesional
biopsy histopathological features were suggestive of histoid leprosy. A
diagnosis of histoid leprosy was made. Child was given MB-MDT WHO regimen
comprising of rifampicin 300 mg and clofazamine 100mg once monthly and daily
Dapsone 25 mg with clofazamine 50 mg twice weekly for 2 years. After 10 months,
majority of the lesions subsided. Child was under regular follow up.
DISCUSSION
Histoid Leprosy
(HL) is a well-recognized clinical entity and is an expression of
multi-bacillary (MB) leprosy. It is characterized by typical cutaneous nodules
or subcutaneous plaques present over apparently normal skin with unique
histopathology and characteristic bacterial morphology [1,2,5]. Histoid leprosy
may appear de-novo or as a manifestation of drug resistance following irregular
or inadequate treatment with dapsone monotherapy [6,7]. Histoid leprosy mimics
several dermatological conditions like xanthoma, sarcoid, neurofibroma, dermatofibroma
and post-kala-azar dermal leishmaniasis [5]. A few cases of HL masquerading as
xanthomas have been reported in the literature [8,9]. The presence of typical
spindle shaped histiocytes, foam cells and AFB differentiates this condition
from other conditions.
Though, the
first case was thoroughly evaluated in USA histopathologically with IHC
markers, simple investigation like skin smears for AFB was not done.
Approximately 1-1.5 million people visit USA every year from India and other
south eastern countries, which are known endemic areas for leprosy. When the
patients from these areas present with nodular lesions or hypopigmented
patches, leprosy should be considered as one of the differential diagnosis.
Knowledge and awareness of leprosy are essential for all dermatologists for
early diagnosis and prevention of disabilities. Skin smear examination is still
a gold standard bed side investigation for diagnosis of lepromatous leprosy and
its variants like histoid leprosy.
CONCLUSION
Though leprosy is no longer a public health
problem in majority of the countries in the world, cases of multi-bacillary
forms of leprosy have been frequently encountered in post-elimination-era.
Knowledge and awareness of leprosy are
essential for all dermatologists and physicians for early diagnosis and
management to prevent permanent disabilities.
1.
Wade HW
(1960) The histoid leproma. Int J Lepr Other Mycobact Dis 28: 469.
2.
Sehgal VN
(2016) Histoid leprosy. In: Bhushan kumar, Hemanth kumar kar, editors. IAL
Textbook of Leprosy. 2nd Edn. New Delhi: Ajanta Ltd., pp: 268-277.
3.
Kaur I,
Dogra S, De D, Saikia UN (2009) Histoid leprosy: A retrospective study of 40
cases from India. Br J Dermatol 160: 305-310.
4.
Mathur M,
Jha A, Joshi R, Wagle R (2017) Histoid leprosy: A retrospective
clinicopathological study from central Nepal. Int J Dermatol 56: 664-668.
5.
Gupta SK
(2015) Histoid leprosy: Review of the literature. Int J Dermatol 54: 1283-1288.
6.
Pandey P,
Suresh MSM, Dey VK (2015) De novo
histoid leprosy. Indian J Dermatol 60: 525.
7.
Bhat YJ,
Hassan I, Yaseen A, Wani R (2015) De novo
histoid leprosy: A case report from a post-elimination area. Indian J Dermatol
60: 214.
8.
Monga P,
Mehta V, Balachandran C, Mathew M (2008) Wade histoid leprosy masquerading as
eruptive xanthomas. Dermatol Online J 14: 21.
9.
Thappa
DM, Karthikeyan K, Vijaikumar M, Laxmisha C (2001) Histoid leprosy masquerading
as tuberous xanthomas. Indian J Lepr 73: 353-358.
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