Should we persist with the term Lazarine leprosy? A historical perspective

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1© 2022 Indian Journal of Dermatology, Venereology and Leprology - Published by Scientic Scholar
Introducon
Leprosy is a chronic infectious disease caused by Mycobacterium
leprae. The clinical spectrum varies from tuberculoid to
lepromatous depending on host immunity. Lazarine leprosy
constitutes an unusual presentation of leprosy.1
The term ‘Lazarine’ is derived from the name of a beggar,
Lazarus in the Bible. According to medieval tradition,
he suffered from leprosy, although not mentioned so in
the Bible.2 The literature concerning Lazarine leprosy is
confusing as there is considerable overlap between the use of
terms Lazarine leprosy and Lucio phenomenon. Additionally,
the term Lazarine leprosy has been used to describe other
ulcerative phenomena in leprosy, especially in malnourished
patients, and as a manifestation of type 1 and type 2 lepra
reactions. In this narrative review, we explore the context and
clinical scenarios in which the term ‘Lazarine leprosy’ has
been used in the literature. The articles were initially retrieved
by PubMed search using the term ‘Lazarine leprosy’. More
articles were accessed using cross-references of the retrieved
articles. After analysing these sources, we present our
opinions on the relevance of the term ‘Lazarine leprosy’.
Lucio phenomenon described as Lazarine leprosy
Ladislao de la Pascua (1844) is credited with describing
Lazarine leprosy or the ‘spotted disease’ for the rst time.3 In
1852, Lucio and Alvarado described it as a form of ulcerating
disease occurring in diffuse non-nodular leprosy.4 Frequent
reactional episodes occurred with scarlet spots that
subsequently darkened and ulcerated, leaving atrophic and
hypochromic scars with a hyperpigmented border. In 1948,
Latapi and Zamora added some more features to the original
description by Lucio and Alvarado and renamed it as Lucio
phenomenon occurring in pure primitive diffuse leprosy.5,6
What Lucio and Alvarado described as the “disease of St.
Lazarus” was probably renamed as Lucio phenomenon later.
This probably resulted in the interchangeable use of the
terms ‘Lazarine leprosy’ and ‘Lucio phenomenon’ by several
subsequent authors.
Lazarine leprosy in malnourished paents
Skinsnes and Higa (1976) suggested that severe ulcerative
phenomena occurring in lepromatous leprosy may be triggered
by an infection in the presence of protein malnutrition with
resultant immunological breakdown.2 Protein malnutrition
was suggested as the major cause of ulceration in these
patients. Hypoproteinemia impairs both cellular and humoral
immunity, increasing the susceptibility to infections by
pathogens, such as streptococci and staphylococci. Intense
tissue oedema secondary to reduced osmotic pressure
increases the probability of ulceration. Strobel et al. (1979)
reported cases of lepromatous leprosy with extensive
ulceration and cachexia and suggested the use of the
terms ‘Lucio phenomenon’ or ‘Lazarine leprosy’ for such
cases.7 Ramu and Dharmendra (1978) sought to differentiate
Lazarine leprosy from Lucio phenomenon.8 They suggested
that Lazarine leprosy occurs near the tuberculoid end of
borderline leprosy in debilitated patients.
Ulcerated type 1 reacon reported as Lazarine
leprosy
In 1930, at the fth international congress of leprosy, it was
concluded that Lazarine leprosy may occur in the tuberculoid
pole because of high inammation and in the lepromatous pole
because of high bacillary load.9 Bhat et al. (2013) reported a
case of ulcerating type 1 lepra reaction in a HIV+ patient with
immune reconstitution inammatory syndrome, mimicking
Lazarine leprosy.10 Sunandini et al. (2015) reported two cases of
Should we persist with the term Lazarine leprosy?
A historical perspective
Neelakandhan Asokan, Vijesh Valsalan
Department of Dermatology & Venereology, Government Medical College, Thrissur, Kerala, India.
How to cite this article: Asokan N, Valsalan V. Should we persist with the term Lazarine leprosy? A historical perspective. Indian J Dermatol Venereol
Leprol doi: 10.25259/IJDVL_1022_2021
Corresponding author: Dr. Neelakandhan Asokan, Department of Dermatology & Venereology, Government Medical College, Thrissur, Kerala, India.
asokann65@gmail.com
Received: October, 2021 Accepted: May, 2022 EPub Ahead of Print: August, 2022 Published: ***
DOI: 10.25259/IJDVL_1022_2021 PMID: ***
History
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Indian Journal of Dermatology, Venereology and Leprology | Volume xx | Issue xx | Month 2022
2
Asokan, et al. Historical perspective on Lazarine leprosy
ulcerating type 1 reaction (one in borderline tuberculoid leprosy,
and another in borderline lepromatous leprosy) as Lazarine
leprosy.11 Both had no underlying debilitating conditions.
Tripathi et al. (2018) described a case of severe ulcerated
type 1 reaction on the face of a lady as Lazarine leprosy.
They sought to differentiate it from the Lucio phenomenon,
another condition described as Lazarine leprosy in previous
literature.12 Wankhade et al. (2020) reported a case of severe
ulcerative type 1 reaction as Lazarine leprosy.13 In their
discussion, they mentioned about the Lucio phenomenon
being reported earlier as Lazarine leprosy and the role of
protein malnutrition as postulated by Skinses and Higa (1976).
Ulcerated type 2 reacon reported as Lazarine leprosy
Cochrane described Lazarine leprosy as a chronic progressive
form of ulcerating erythema nodosum leprosum associated with
severe systemic illness.14 After a detailed discussion, Dharmendra
and Desikan (1985) acknowledged that reports on Lazarine
leprosy are conicting, but most of the reported cases represent
severe ulcerated erythema nodosum leprosum.15 Strobel et al.
(1981) reported a case of ulcerated erythema nodosum leprosum
as Lazarine leprosy.16 Fogagnolo (2007) pointed out the
inappropriate use of the term Lucio phenomenon for ulcerating
erythema nodosum leprosum and described the differentiating
features between these two conditions.6
Use of the term Lazarine leprosy in other situaons
In 2004, Nanda et al. reported two cases of borderline tuberculoid
leprosy both with two skin lesions each and a bacteriological
index of 2 +, who developed spontaneous ulceration of the
existing lesions without any other features of lepra reactions,
during multibacillary multidrug therapy for leprosy.17 They
described these cases as unique in that they were not in the
lepromatous spectrum with high bacillary load; did not display
features of lepra reactions, including the Lucio phenomenon;
nor had marked malnutrition. A summary of different clinical
conditions described as Lazarine leprosy is given in Table 1.
Discussion
There are two characters mentioned as Lazarus (a beggar
having sores [Luke 16:19–31] and the Lazarus of Bethany
[John 11:1–44]) in the Bible, with occasional conation
between the two.18 Interestingly, even after several centuries,
a similar conation exists in medical literature too, regarding
Lazarine leprosy.
Skinsnes and Higa (1976) had described how the use of term
‘disease of St. Lazarus’ by Lucio and Alvarado in their paper,
resulted in the interchangeable use of the terms ‘Lazarine
leprosy’ and ‘Lucio phenomenon’ by subsequent authors.2
After analysing the paper by Lucio and Alvarado, they
inferred that the former used the term ‘spotted form’ to denote
the Lucio phenomenon, and used ‘the disease of St. Lazarus’
to denote leprosy in a general sense. But, unfortunately,
subsequent papers probably missed this distinction and used
the term Lazarine leprosy for Lucio phenomenon and for
several other ulcerative phenomena in leprosy including type
1 and type 2 lepra reactions. Skinsnes and Higa quoted Pardo-
Castello and Pineyro (1948) and Wade (1949) who opined
against the use of the term Lazarine leprosy.2,19,20 Despite this,
Table 1: A summary of di󰀨erent clinical conditions described as ‘Lazarine leprosy’
Clinical conditions Authors Year Country Patient details Remarks
Lucio phenomenon Lucio, Alvarado41852 Mexico Not available Described as ‘spotted disease’
Ulcerated type 1 lepra
reaction
Bhat et al.10 2013 India The patient on HAART
developed BT lesions which
subsequently ulcerated.
As a part of immune reconstitution
inammatory syndrome in HIV
Sunandini et al.11 2015 India One patient in BT, and
another in BL
Two cases of Type 1 reaction with
ulceration
Tripathy et al.12 2018 India BT patient with facial
lesions
Facial Lazarine leprosy in an
immunocompetent patient without
underlying malnutrition
Wankhade et al.13 2020 India BT patient with features of
Type 1 reaction and intense
ulceration
Nil
In malnourished patients Skinsnes and Higa21976 China An experimental study in rats Suggested that severe ulcerative
phenomenon occurring in lepromatous
leprosy may be caused by malnutrition
Strobel et al.71979 France In patients of lepromatous
leprosy with cachexia
Nil
Ulcerated type 2 lepra
reaction
Strobel et al.16 1981 France One patient with ulcerating
erythema nodosum leprosum
Used the term Lazarine leprosy as an
alternative term for ulcerating ENL
Other clinical
presentations in leprosy
Nanda et al.17 2004 India Two patients in BT
spectrum
Both had BI 2 +, and tissue oedema; One
patient was poorly nourished; No evidence
of severe malnutrition, infection, or
defence breakdown in both
HAART: Highly active antiretroviral therapy; BT: Borderline tuberculoid leprosy; HIV: Human immunodeciency virus disease; BL: Borderline lepromatous leprosy;
ENL: Erythema nodosum leprosum; BI: Bacteriological index

3Indian Journal of Dermatology, Venereology and Leprology | Volume xx | Issue xx | Month 2022
Asokan, et al. Historical perspective on Lazarine leprosy
there has been continued use of the term ‘Lazarine leprosy’ to
describe a variety of ulcerative phenomena - except trophic
ulcers - in leprosy, which differ widely in their pathogenesis,
clinical features and management. The only common feature
among all these reports is marked ‘leprous’ ulceration.
Thapa (2005) opined that spontaneous ulceration may
occur in histoid lesions or lesions with high bacillary load,
even in the absence of features denoting type 1 or type 2
lepra reactions or the Lucio phenomenon.21 He postulated
that acute exacerbation of the disease, in which bacterial
multiplication surpassed the macrophage population is the
likely pathogenesis in such cases.22 Thapa suggested to refrain
from using the term ‘Lazarine leprosy’ even for such cases.21
Recently, an increasing number of ulcerated type 1 lepra
reactions are being reported as Lazarine leprosy.10–13
However, all leprous ulcers are not termed ‘Lazarine’. There
are several reports of Lucio phenomenon, without describing
them as Lazarine leprosy.23–28 Similarly, several articles do
not refer to cases of ulcerating type 1 or type 2 lepra reactions
as Lazarine leprosy.
Conclusion
Given the considerable discordance in the nomenclature, it is
better to restrict the use of term Lazarine leprosy to describe
those rare instances of spontaneous ulceration in leprosy
associated with malnutrition, as suggested by Skinsnes and
Higa (1976).2 Ulcerating type 1 lepra reaction, ulcerating
type 2 lepra reaction and Lucio phenomenon occurring in
Lucio leprosy may better be described as such, instead of as
Lazarine leprosy, as they possess distinct pathogenetic and
clinical features.
Financial support and sponsorship
Nil.
Conict of interest
None.
References
1. Sardana K, Bhushan P, Khurana A. The Disease. In. Sardana K,
Khurana A, ed. Jopling’s Handbook of Leprosy. 6th ed. New Delhi:
CBS Publishers & Distributors (P) Ltd; 2020. p. 6–58.
2. Skinsnes LK, Higa LH. The role of protein malnutrition in the
pathogenesis of ulcerative “Lazarine” leprosy. Int J Lepr other
Mycobact Dis 1976;44:346–58.
3. Vargas-Ocampo F. Diffuse leprosy of Lucio and Latapí: A histologic
study. Lepr Rev 2007;78:248–60.
4. Lucio R, Alvarado I. Opusculo sobre el mal de San Lazaro o
elephantiasis de los Griegos. México: Murguía e Cia; 1852. as cited by
Fogagnolo L, de Souza EM, Cintra ML, Velho PENF. Vasculonecrotic
reactions in leprosy. Braz J Infect Dis 2007;11:378–82.
5. Latapi F, Zamora AC. The ‘spotted’ leprosy of Lucio. An introduction to
its clinical and histological study. Int J Lepr 1948;16:421–30. As cited by
Fogagnolo L, de Souza EM, Cintra ML, Velho PENF. Vasculonecrotic
reactions in leprosy. Braz J Infect Dis 2007;11:378–82.
6. Fogagnolo L, de Souza EM, Cintra ML, Velho PENF. Vasculonecrotic
reactions in leprosy. Braz J Infect Dis 2007;11:378–82.
7. Strobel M, Ndiaye B, Carayon A. [Lepromatous leprosy with extensive
ulcerations and cachexia. The Lucio phenomenon? Lazarine leprosy?]
Acta Leprol 1979:331–3.
8. Ramu G, Dharmendra. Acute exacerbations (reactions) in leprosy,
In: Dharmendra, ed.Leprosy, Vol 1. 1st ed. Bombay: Kothari Medical
Publishing House; 1978. p. 108–39.
9. Pardo–Castello V, Caballero GM. Lazarine leprosy: A peculiar
monosymptomatic form of leprosy. Arch Derm Syph 1931;23:1–11. as
cited by Wankhade V, Shah V, Singh RP, Bhat D. Lazarine leprosy: A
unique phenomenon of leprosy. Int J Mycobacteriol 2020;9:329–31.
10. Bhat R, Pinto M, Dandakeri S, Kambil S. Ulcerating type 1 lepra
reaction mimicking Lazarine leprosy: An unusual presentation of
immune reconstitution inammatory syndrome in an HIV-infected
patient. Int J STD AIDS 2013;24:992–4.
11. Sunandini PA, Prasad PG, Chalam KV, Padmasri Y. Type 1 lepra
reaction with ulceration (Lazarine leprosy) - Two interesting case
reports. IOSR J Dent Med Sci 2015;14:22–5.
12. Tripathy T, Panda M, Kar BR, Thakur TK, Singh BSTP. Facial
Lazarine leprosy in post-elimination era: A case report. Indian J Lepr
2018;90:313–8.
13. Wankhade V, Shah V, Singh RP, Bhat D. Lazarine leprosy: A unique
phenomenon of leprosy. Int J Mycobacteriol 2020;9:329–31.
14. Cochrane RG. Complicating conditions due to leprosy In: Cochrane
RG, ed. Leprosy in theory and practice. 2nd ed. Bristol: John Wright
and Sons Ltd; 1964. p. 152–82.
15. Dharmendra, Desikan KV. Mechanisms of reactions. In: Dharmendra,
ed. Leprosy Vol 2. Bombay: Samant and Company; 1985. p. 984–98.
16. Strobel M, Ndiaye B, Marchand JP, Stach JL, Foumoux F. [Leprosy tests:
Diagnostic problems (apropos of 2 cases)]. Acta Leprol 1981;83:11–9.
17. Nanda S, Bansal S, Grover C, Garg V, Reddy BSN. Lazarine leprosy–
revisited? Indian J Lepr 2004;76:351–4.
18. Wikipedia contributors. Rich man and Lazarus. In: Wikipedia
[Internet]. 2021. Available from: https://en.wikipedia.org/w/index.
php?title=Rich_man_and_Lazarus&oldid=1037461952. Last accessed
15.10.2021.
19. Pardo-Castello V, Pineyro R. Lazarine leprosy. Its position in the present
classication. Int J Lepr 1949;17:65–72. as cited by Skinsnes LK, Higa
LH. The role of protein malnutrition in the pathogenesis of ulcerative
“Lazarine” leprosy. Int J Lepr other Mycobact Dis 1976;44:346–58.
20. Wade HW. The Lucio and Lazarine forms of leprosy. Int J Lepr
1949;17:95–102. as cited by Skinsnes LK, Higa LH. The role of protein
malnutrition in the pathogenesis of ulcerative “Lazarine” leprosy. Int J
Lepr other Mycobact Dis 1976;44:346–58.
21. Thappa DM. What is Lazarine leprosy? Is it a separate entity? Indian J
Lepr 2005;77:179–81.
22. Job CK. Pathology of leprosy. In: Hastings RC, ed. Leprosy. 2nd ed.
London: Churchill Livingstone; 1994. p. 193–224.
23. Ranugha PSS, Chandrashekar L, Kumari R, Thappa DM, Badhe B. Is
it lucio phenomenon or necrotic erythema nodosum leprosum? Indian J
Dermatol 2013;58:160.
24. Sharma P, Kumar A, Tuknayat A, Thami GP, Kundu R. Lucio
phenomenon: A rare presentation of Hansen’s disease. J Clin Aesthet
Dermatol 2019;12:35–8.
25. Prem Anand P, Oommen N, Sunil S, Deepa MS, Potturu M. Pretty
leprosy: Another face of Hansen’s disease! A review. Egypt J Chest Dis
Tuberc 2014;63:1087–90.
26. Herath S, Navinan MR, Liyanage I, Rathnayaka N, Yudhishdran J,
Fernando J, et al. Lucio’s phenomenon, an uncommon occurrence
among leprosy patients in Sri Lanka. BMC Res Notes 2015;8:672.
27. Saúl A, Novales J. [Lucio-Latapí leprosy and the Lucio phenomenon].
Acta Leprol 1983;1:115–32.
28. Fenniche S, Benmously R, Sa M, Daoud L, Debbiche A, Ben Ayed M,
et al. [Late-occurring cutaneous vasculitis after successful treatment of
diffuse lepromatous leprosy: Lucio’s phenomenon]. Med Trop (Mars)
2007;67:65–8.