Egyptian Journal of Chest Diseases and Tuberculosis (2014) 63, 1087–1090

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 Hansen’s 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 infiltrated 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.
ª 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 ª 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

. . and blood vessel walls inva- (Spanish word). . Columbia variety of leprosy is sometimes called ‘‘pretty leprosy’’. septum perforation and destruc- tion of the nasal cartilages. The prominent infiltration of leprosy. . . . . . . . . . . This tends to iron out Hansen’s disease for leprosy [7]. Clinical features and manifestations. . According to Vagbhata (600 AD). . Several LP trig- actually a clinical variety of lepromatous leprosy [8. . . . . . . . gering factors that have been suggested are streptococcal. . . Hansen’s disease or Hanseniasis [2–6]. Leprosy bonita/pretty leprosy/Virchowian Hanseniasis [24–27] refers to the initial loss of wrinkling in It is primarily seen in Mexico (23% cases). . Prem Anand et al. Later on in 1852. . . giving an ichthyosiform appearance to some areas such as the legs [14. . . Flaccid skin out [24]. . . . . . Lucio and suggested by various authors. . The central portion of the forehead (fron- talis muscle) is more affected than the lateral portions. . . . sion by Hansen’s bacillus. . respiratory infections. . . . . ischemia and tissue necrosis. . . . . leprae in endothelial cells and enhanced is an important cause of crippling deformities [1]. . . . . . . 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. . . . calling these lesions ‘Fenómeno According to International literature. . . . upon which necrotizing lesions develop. . . drugs such as iodide. . . . . . . . . . three criteria are de Lucio or erythema necrotisans’. . The sensory loss on the forehead can be quite marked (since the skin is relatively cool) but at the hair- line. . . . .13]. . . . a disease of unparal. . . the diffuse Rica. (1948). .23]. . . . . 1088 Investigations . puffy and quite rare in the rest of the world. This associated with originated in India [30]. Over time all skin become was first described in Mexico by Doctor Rafael Lucio in 1853 flaccid and atrophic. . . eyelashes and body hair. . . . . Arthrus Phenomenon and Schwartzman Phenomenon are also arine leprosy’ [8] for the first time. . . . . . . . . cryp- Latapı́ (1938) re-discovered it and reported with Zamora [26] tococcal. . . . . . Kushtha. [10] Shiny [11] myxedoema. . . . . . 1089 Introduction Leprosy is known as death before death. . . in particular that of ear lobe. This sign is quite characteristic for leprosy and was first described by Monrad–Krohn. . . . . . . . Leprosy is exposure of mycobacterial antigen to circulating antibodies. . . . . but the origin is still unknown. . . . . . the name ‘‘Kush- tha’’ was derived from ‘‘Kushnati’’ which means ‘‘eating Etiopathogenesis away’’ in Sanskrit. . . . one of the Latin American Countries [8. . . less common in gulf coast [15]. . . . . adopted as a rule for Lucio phenomenon definition: cutaneous toid appearance of the skin made us call it as Lepra Bonita ulceration. . . . . . . compared with myxodema.26]. . . . . . . . . . . . . The disease leprosy was first described around 600 BC. . . . . the same as ‘spotted leprosy of Lucio’. . . . . . . . . . . . . . 1089 References . . . . . . . . . . . . . . This sort of Hansen disease red and legs become edematous. . . .25] to the older patients. . . . . . . the wrinkles in the skin of face. . . A case of lepromatous leprosy with Lucio’s phenomenon leled historical stigma and fear that has earned the appellation was reported recently in India [31]. The clinical sine qua non criterion History shows evidence of a debate in 1960–1970s about the is diffuse non nodular infiltration of the skin that has been choice of selecting an appropriate name for this disease viz. .9. .. . . included instructions for causes vascular thrombosis. . stated in the Vedas inflammatory reactions and changes in the coagulation system written as early as 1400 BC in India. . . . . . . . .1088 P.23. . since it free replication of M. . Latapı́ and Chévez-Zamora explained that the chief presentation of this variety of lesion was diffuse generalized cutaneous infiltra. . . . In Mexico. . It is triggering factor and precipitate the reaction. . . The laws of Manu. . . [16]. . Infiltration of the mucosa leads to chronic rhinitis with epistaxis. . . There will not be any Epidemiology nodule formation. . lepra. . .17]. . . . . . .17. Frenken named chaulmoogra oil and pregnancy [28]. . Throughout The most accepted pathogenesis for Lucio’s phenomenon is history. lead- the prevention of leprosy (Fig. common in Costa some patients with diffuse disease [20]. Later and Brazil [14] and. . . . . . . . it as ‘diffuse leprosy of Lucio and Latapı´’ in 1963 [10]. . vascular thrombosis. . . . . . . . . 1089 Treatment . . . . The upper incisors become loose and often drop Figure 1 Diffuse lepromatous leprosy in late stage. . . . . . . . . . . . . . . . . . . There is often madarosis. one of the oldest diseases of mankind which most probably resulting in vasculitis and infarction. . leprosy has been feared and misunderstood. . . 1). . . The tongue is thickened and there may be hoarseness. meaning ‘pretty leprosy’ [12. . . In 1844 Ladislao de la Pascua described the ‘spotted or laz. . ing to the histopathological features of the phenomenon [32]. restoring a youthful appear- ance [14. there tends to be an abrupt increase in the sensitivity to pinprick. . . . Clinical features and manifestations tion (pure and primitive diffuse lepromatosis). . . . . complete alopecia of eyebrows. . But it is on progression of disease hands become swollen. . Intercurrent infections may be a Alvarado studied and published it with the same names. .

22]. clofazimine. . II Edition. later becoming necrotic with mation and education should be maintained to keep both the scabs. Hanseniasis: The polar concept as it stands today. Pause and W. Oxford plementemia (Fig. [22] Lucio R. [14] Enrico Nunzi. Novales. 8 (1969) 40–43. cryoglobulins. Histopathologically there is wide 3159. Dis. lepra or Hansen’s disease: Choice of no lepromas but there is diffuse infiltration of the whole skin appropriate name. tha. Lucio-Latapı́ Leprosy. Dermatol. tion and neutrophilic infiltration [19. forearms. L. 2012 Farlex. Clinical and histological variations among thirty patients with Lucio’s phenomenon and pure primitive lepromatosis (Lapatis lepromatosis). Kawano. Vargas-Ocampo. J. México: 53. tological features consistent with Lucio phenomena includes [7] A. Int J Though it has been plaguing us since at least 2000 BC. J. third ed. positive rheumatoid factor. false positive serological Companions Dermatology. Clinical Companions Dermatology by W with its legacy of stigmatization. [4] R. 1 (3) (1983) 115–132. arthritis and nephritis. ‘‘Hanseniasis’’. hands. Dermatol Ver Mex. Bope.P. Int. 2013 The systemic manifestations of LP include hepatomegaly. Firth. bly belonging to a particular strain named Mycobacterium Other. splenomegaly. Cochrane.19]. 1997. [8] A. Moreover when a dentist encounters patients with vessels especially of the dermohypodermic union and of the these facial changes this rare entity should also be considered hypodermis. Ladhani. ISBN: 3540594523. endothelial proliferation of the med- [9] Thappa. Med. proba. Jerskey. Diffuse leprosy of Lucio and Latapı́: A The Lab findings observed in Lucio’s phenomenon are ane. 4 (2002) However it has to be differentiated from erythema nodosum.M. Lucio-Latapı́ leprosy and the Lucio epidermal necrois. the trunk and face. Hyg. 2) [31]. mixed type [12] Segen’s Medical Dictionaryª. Leprology. Thieme PUBLIC. Warrell.E. atlas. J. 2009. Am. Mycobact. marked thickening of vessel walls to the point of obliteration. reactions for syphilis. 36 (1997) 561–572. Burgdorf. Rea.P. Dermatology. Nogueira. hypergammaglobunemia. affecting in ascending order of public and health professionals aware of how to recognize frequency: feet. rarely. R. Opusculo sobre el mal de San Lazaro o are reports that go from balanced meals and local hygiene only elefantiasis de Los Griegos (1851). Brazil Dermatol. Murguı́a y Cı́a. 38 (1970) 207–209. an elevated erythrocyte sedimentation rate. R. Leprosy for medical practitioners and paramedical workers. There is no specific treatment for Lucio’s phenomenon. Angiogenesis. Trop. non is very rare in the Indian subcontinent also we should have [21] Mario Magana. 1 ous vessels are characterized by endothelial proliferation and (1952) 999–1008. Mexico. infiltration by macrophages filled with acid-fast bacilli. Int. the new official name for leprosy in colonization of endothelial cells by acid fast bacilli. Rev. 278–292. [2] A. thighs. Treatment [15] Edward T. 96 (1952) 3157– and is multibacillary [14]. Figure 2 Highly bacilliferous diffuse lepromatous leprosy with no nodule formation. pp 111–114. [3] K. They are frequently painful. J. lepromatosis [14. Timothy M. Lepr. 72 (2005) 169–188. 22 or the use of isolated drugs such as dapsone. and deal with leprosy cases. by disseminated intravascular coagulation and/or septicemia. In defense of the name ‘‘leprosy’’. Nomenclature for leprosy. Dermatologia. Pereira.A. D. In References advanced cases the skin become atropic like a cigarette paper. leaving atrophic and stellar scars [18. phenomenon: a case report and review of the literature.D. Other. Lucio’s Leprosy and humans have a long bitter relationship. Rarely it can be fatal [8]. Gramberg. necrotising vasculitis of the small vessels phenomenon. Leprosy disabilities: The impact of multidrug Investigations therapy (MDT). The name leprosy. John D. Saul. It may cause death Interamericana-Mc Graw-Hill. Mycobact. Kellerman.Pretty leprosy 1089 A peculiar type of lepra reaction named Lucio’s phenome. W. Leprosy. mia. Burgdorf. The subject usually shows no response to lepromin skin test.R. J.23]. [24] S. circulating immune complexes and hypocom. histologic study. 114. Int. Pause. [1] S. [16] R. legs. Lepr. [13] D. [23] T. 78 (3) (2007) 248–260. 2012.S. Areenas. 11–15. [6] F. Textbook of Medicine. and ium sized vessels of the mid dermis with passive venous conges. barely understand it 4000 years later and we are still wrestling [20] Thieme PUBLIC. of the superficial dermis. Vascular patterns affecting all cutane. Acta Leprol. Alvorado. M. Lepr.C. Lendrum. hypoalbumi.. Textbook Book of Dermatology. Sherry. arms and. 48 (1980) 318–321. J. Mycobact. Otto Braun Falco. M. Alvarado I (1852) Opúsculo sobre el mal de San Lazaro o elefanciasis de los Griegos: M. diagnostico y tratameino.21. Yawalkar.B. [10] F. 44 (2005) 566–571. Klin. Although Lucio’s phenome. Leprosy: A Practical Guide. Current management of leprosy reactions. Venerology. Dis. Rotberg. Clinical nemia. J. fever. ulcerations and scars. first ed.S. (1978) 93–101. Lepr. The his. Other. 36 (1968) 227–229. Dis. Costa.. Int. Dermatol. Cesare Massone. There [18] L. Sherry. lidomide and multidrug therapy. Clinically this vasculitis is represented by well. [17] Roberto Arenas. Conn’s Current Therapy. and Thrombosis [10]. index of suspicion to diagnose and differentiate it from other non [27–29] or necrotic erythema occurs which is vasculitis of entities. C.G. 2012. R. [11] W. Rabello. ischemic Sao Paulo. Springer. [5] F.. Vascular ectasia. Ned Tijdschr Geneeskd. Lepr. [19] I. Int. Rick D. we Dermatol.H. L. Cox. Eighth revised ed. Julieta Ruiz-Esmenjauid. Rotberg. in the differential diagnosis. Int. J. A high level of community infor- shaped erythematous spots.

Roncada. E. cases seen in Johor. Dermatol. 988.1090 P. Tey.M. Basic Appl. paramedical workers. Med. report. Med. 79 (2004) 205–210. Iber. Lat. L. An. Am. Dermatol. K. L.V. Neto. L. Lepr. 3 (2013) 325–330. Olveria. Manual of Tropical Dermatology.H.D. Parish. Lucio’s phenomenon: A rare case histologic study. Revisiting Lucio’s leprosy and [32] R. 33 (2005) C.G. Abreu.C. case reported in Brazil. I.E. [27] F.S. Tiezzi. Sacchidanand. 1984. 78 (2007) 248–260. Leprosy-for medical practitioners and necrosante) na gestação. Ortigosa. Diffuse leprosy of Lucio and Latapı́: A [31] M.M. M. Int.A. Prem Anand et al. Helmer. 2002. [25] J. Bras Dermatol. Vargas-Ocampo. Monteiro. [28] L.E. Santamaria. seventh ed. New York. J. J. J. Fenômeno de Lúcio (eritema [30] Yawalkar S. Lucio’s phenomenon: a report of three Springer Verlag. Spinelli.C.M.M. Rev. Kavya. Berlin. Lucio’s phenomenon. L. Sci. Kucharski-Esmanhoto. J.R. Cutan.C.M. Bras. Lucio’s phenomenonAnother 125–133. Azulay-Abulafia. J. Fleishfresser. M. Pettit. 48 (2009) 984– [26] K.F. [29] S. 87 (2) (2012) 296–300. Choon. . Malaysia Int.A. S.