Egyptian Journal of Chest Diseases and Tuberculosis (2014) 63, 10871090

H O S T E D BY
The Egyptian Society of Chest Diseases and Tuberculosis

Egyptian Journal of Chest Diseases and Tuberculosis

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REVIEW

Pretty leprosy: Another face of Hansens disease!

A review
a,*
P. Prem Anand , Neetu Oommen b, S. Sunil b, M.S. Deepa c, Mythri Potturu d

a
Department of Oral & Maxillofacial Pathology, School of Dentistry, Amrita Institute of Medical Sciences and Research,
Cochin, Kerala, India
b
Department of Oral & Maxillofacial Pathology, Azeezia College of Dental Sciences & Research, Kerala, India
c
Department of Oral Medicine and Radiology, Azeezia Dental College and Research, Kollam, Kerala, India
d
Oral & Maxillofacial Pathology, Vipula Care Hospital, Vijayawada, Andhra Pradesh, India

Received 31 March 2014; accepted 17 April 2014

Available online 27 August 2014

KEYWORDS Abstract Pretty leprosy is one of the rare severe forms of lepromatous leprosy. It is a reaction pattern
Pretty leprosy; that occurs in untreated pure primitive diffuse lepromatous leprosy or lepromatous leprosy. The skin
Beautiful leprosy of the patient is diffusely inltrated and shiny, so that natural wrinkles are obliterated. It generates a
Lucioleprosy; moist and myxoedematous complexion imparting a healthy aspect to the patient. Thus the disease got
Lepra Bonita the name. In this review we have reviewed almost all available literature to point more light toward the
clinical variant pretty leprosy. A search was made in PubMed central to know the availability of infor-
mation about pretty leprosy, but the response was nil for pretty leprosy. 37 articles were spotted in
PubMed media on Lucioleprosy; 3 review articles are present and the rest were case reports.
2014 The Egyptian Society of Chest Diseases and Tuberculosis. Production and hosting by Elsevier
B.V. Open access under CC BY-NC-ND license.

Contents

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1088
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1088
Etiopathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1088

* Corresponding author. Address: Department of Oral & Maxillofa-

cial Pathology, School of Dentistry, Amrita Institute of Medical
Sciences & Research, Kerala 41, India. Tel.: +91 9387113003, +91
9745213825.
E-mail address: premanandp@aims.amrita.edu (P. Prem Anand).
Peer review under responsibility of The Egyptian Society of Chest
Diseases and Tuberculosis.
0422-7638 2014 The Egyptian Society of Chest Diseases and Tuberculosis. Production and hosting by Elsevier B.V.
Open access under CC BY-NC-ND license. http://dx.doi.org/10.1016/j.ejcdt.2014.04.005
1088 P. Prem Anand et al.

Clinical features and manifestations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1088

Investigations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1089
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1089
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1089

Introduction

Leprosy is known as death before death; a disease of unparal- [16]. A case of lepromatous leprosy with Lucios phenomenon
leled historical stigma and fear that has earned the appellation was reported recently in India [31].
Kushtha. According to Vagbhata (600 AD), the name Kush-
tha was derived from Kushnati which means eating Etiopathogenesis
away in Sanskrit. The disease leprosy was rst described
around 600 BC., but the origin is still unknown. Throughout The most accepted pathogenesis for Lucios phenomenon is
history, leprosy has been feared and misunderstood, since it free replication of M. leprae in endothelial cells and enhanced
is an important cause of crippling deformities [1]. Leprosy is exposure of mycobacterial antigen to circulating antibodies,
one of the oldest diseases of mankind which most probably resulting in vasculitis and infarction. This associated with
originated in India [30]. The laws of Manu, stated in the Vedas inammatory reactions and changes in the coagulation system
written as early as 1400 BC in India, included instructions for causes vascular thrombosis, ischemia and tissue necrosis, lead-
the prevention of leprosy (Fig. 1). ing to the histopathological features of the phenomenon [32].
In 1844 Ladislao de la Pascua described the spotted or laz- Arthrus Phenomenon and Schwartzman Phenomenon are also
arine leprosy [8] for the rst time. Later on in 1852, Lucio and suggested by various authors. Intercurrent infections may be a
Alvarado studied and published it with the same names. It is triggering factor and precipitate the reaction. Several LP trig-
actually a clinical variety of lepromatous leprosy [8,9,26]. gering factors that have been suggested are streptococcal, cryp-
Latap (1938) re-discovered it and reported with Zamora [26] tococcal, respiratory infections, drugs such as iodide,
(1948), the same as spotted leprosy of Lucio. Frenken named chaulmoogra oil and pregnancy [28].
it as diffuse leprosy of Lucio and Latap in 1963 [10]. Latap
and Chevez-Zamora explained that the chief presentation of
this variety of lesion was diffuse generalized cutaneous inltra- Clinical features and manifestations
tion (pure and primitive diffuse lepromatosis), upon which
necrotizing lesions develop, calling these lesions Fenomeno According to International literature, three criteria are
de Lucio or erythema necrotisans. [10] Shiny [11] myxedoema- adopted as a rule for Lucio phenomenon denition: cutaneous
toid appearance of the skin made us call it as Lepra Bonita ulceration, vascular thrombosis, and blood vessel walls inva-
(Spanish word), meaning pretty leprosy [12,17]. sion by Hansens bacillus. The clinical sine qua non criterion
History shows evidence of a debate in 19601970s about the is diffuse non nodular inltration of the skin that has been
choice of selecting an appropriate name for this disease viz. compared with myxodema. The prominent inltration of
leprosy, lepra, Hansens disease or Hanseniasis [26]. A strong auricular lobes makes it appear thick and odematous appear-
movement developed in some countries to substitute the name ance of face makes it appear healthy. This tends to iron out
Hansens disease for leprosy [7]. the wrinkles in the skin of face, restoring a youthful appear-
ance [14,17,23,25] to the older patients. There will not be any
Epidemiology nodule formation. Leprosy bonita/pretty leprosy/Virchowian
Hanseniasis [2427] refers to the initial loss of wrinkling in
It is primarily seen in Mexico (23% cases), common in Costa some patients with diffuse disease [20]. In Mexico, the diffuse
Rica, one of the Latin American Countries [8,13], Columbia variety of leprosy is sometimes called pretty leprosy. Later
and Brazil [14] and; less common in gulf coast [15]. But it is on progression of disease hands become swollen, puffy and
quite rare in the rest of the world. This sort of Hansen disease red and legs become edematous. Over time all skin become
was rst described in Mexico by Doctor Rafael Lucio in 1853 accid and atrophic, giving an ichthyosiform appearance to
some areas such as the legs [14,23].
There is often madarosis, complete alopecia of eyebrows,
eyelashes and body hair. Inltration of the mucosa leads to
chronic rhinitis with epistaxis, septum perforation and destruc-
tion of the nasal cartilages. The tongue is thickened and there
may be hoarseness. The central portion of the forehead (fron-
talis muscle) is more affected than the lateral portions. This
sign is quite characteristic for leprosy and was rst described
by MonradKrohn. The sensory loss on the forehead can be
quite marked (since the skin is relatively cool) but at the hair-
line, there tends to be an abrupt increase in the sensitivity to
pinprick. The upper incisors become loose and often drop
Figure 1 Diffuse lepromatous leprosy in late stage. Flaccid skin out [24].
in particular that of ear lobe.
Pretty leprosy
A peculiar type of lepra reaction named Lucios phenome-
non [2729] or necrotic erythema occurs which is vasculitis of
vessels especially of the dermohypodermic union and of the
hypodermis. Clinically this vasculitis is represented by well-
shaped erythematous spots, later becoming necrotic with
scabs; ulcerations and scars; affecting in ascending order of
frequency: feet, legs, hands, forearms, thighs, arms and, rarely,
the trunk and face, leaving atrophic and stellar scars [18,19].
They are frequently painful. Rarely it can be fatal [8]. In
advanced cases the skin become atropic like a cigarette paper.
Investigations
The subject usually shows no response to lepromin skin test,
no lepromas but there is diffuse inltration of the whole skin
and is multibacillary [14]. Histopathologically there is wide
inltration by macrophages lled with acid-fast bacilli, proba-
bly belonging to a particular strain named Mycobacterium
lepromatosis [14,21,22]. Vascular patterns affecting all cutane-
ous vessels are characterized by endothelial proliferation and
marked thickening of vessel walls to the point of obliteration,
Angiogenesis, Vascular ectasia, and Thrombosis [10]. The his-
tological features consistent with Lucio phenomena includes
colonization of endothelial cells by acid fast bacilli, ischemic
epidermal necrois, necrotising vasculitis of the small vessels
of the supercial dermis, endothelial proliferation of the med-
ium sized vessels of the mid dermis with passive venous conges-
tion and neutrophilic inltration [19,23].
The Lab ndings observed in Lucios phenomenon are ane-
mia, an elevated erythrocyte sedimentation rate, hypoalbumi-
nemia, hypergammaglobunemia, false positive serological
reactions for syphilis, positive rheumatoid factor, mixed type
cryoglobulins, circulating immune complexes and hypocom-
plementemia (Fig. 2) [31].
Treatment
The systemic manifestations of LP include hepatomegaly,
splenomegaly, fever, arthritis and nephritis. It may cause death
by disseminated intravascular coagulation and/or septicemia.
However it has to be differentiated from erythema nodosum.
There is no specic treatment for Lucios phenomenon. There
are reports that go from balanced meals and local hygiene only
or the use of isolated drugs such as dapsone, clofazimine, tha-
lidomide and multidrug therapy.
Leprosy and humans have a long bitter relationship.
Though it has been plaguing us since at least 2000 BC, we
barely understand it 4000 years later and we are still wrestling
with its legacy of stigmatization. Although Lucios phenome-
non is very rare in the Indian subcontinent also we should have
Figure 2 Highly bacilliferous diffuse lepromatous leprosy with
no nodule formation.
1089
index of suspicion to diagnose and differentiate it from other
entities. Moreover when a dentist encounters patients with
these facial changes this rare entity should also be considered
in the differential diagnosis. A high level of community infor-
mation and education should be maintained to keep both the
public and health professionals aware of how to recognize
and deal with leprosy cases.
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