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Leprosy: A glossary
Marcos Virmond, MD, PhD a,, Andrzej Grzybowski, MD, PhD b,c , Luiza Virmond, Mst d
a
Division of Rehabilitation, Instituto Lauro de Souza Lima, Rod Cmt Joao R de Barros, Km 226, Bauru-SP, 17034-971, Brazil
b
Department of Ophthalmology, Poznan City Hospital, ul., Szwajcarska 361-285 Poznan, Poland
c
Department of Ophthalmology, University of Warmia and Mazury, Olsztyn, Poland
d
Department of Internal Medicine, School of Medicine, Pontifical Catholic University, Rua Imaculada Conceio 1155, Prado
Velho, Curitiba, Parana, 80215-901, Brazil
Abstract Leprosy continues to afflict residents from a number of countries in Africa, South America, and
southeast Asia, despite the marked reduction in the number of cases of leprosy worldwide, after the
introduction of the multidrug regimens as recommended by the World Health Organization (WHO-MDT).
With the increasing immigration of individuals from risk areas to Europe and the United States, knowledge
of the basic concepts of leprosy would be helpful to clinicians caring for immigrants in nonendemic areas.
We present a comprehensive, updated, and critical glossary of the most relevant terms related to leprosy.
2015 Published by Elsevier Inc.
http://dx.doi.org/10.1016/j.clindermatol.2014.07.006
0738-081X/ 2015 Published by Elsevier Inc.
Leprosy: A glossary 9
Anhidrosis
Decreased secretion or even absence of sweat. Skin lesions Fig. 1 Blepharochalasis as a common finding in long-standing
lepromatous patients.
in leprosy show marked reduction of sweating as evidenced by
a simple test using 1% ninhydrin paper stamps.5
and nodules, which are usually found in combination with
the typical lesions (Figure 2).
Amyloidosis
Any opacification of the crystalline lens. Side effects caused by the cumulative dose of clofazimine
and a form of ocular toxicity in patients receiving long-
standing antileprosy multidrug therapy.
Chemoprophylaxis
Demyelination
A cell from the immune system specialized in antigen The emergence of multidrug-resistant strains of M leprae
presentation to T cells and activation of the immune response. would pose a serious threat to leprosy control efforts because
control depends mainly on case findings and adequate
treatment with MDT. There is some evidence of emerging
strains in which the bacilli are resistant to dapsone and
Detection rate rifampicin. WHO has developed a global initiative to
monitor drug resistance in leprosy.
The number of new cases of leprosy diagnosed in a given
population at a specific time of a given year, divided by the
population in which the cases occur. The new-case detection
rate is a better indicator of disease than prevalence rate, Ectropion
because it is not affected by the operational aspects of the
control program, such as changing case definitions or An outward turning of the eyelid margin from the
duration of treatment.9 globe (Figure 4).
A patient diagnosed with leprosy with one or more of the A World Health Organization public health strategy
following features: (i) hypopigmented or reddish skin lesions aimed at reducing the proportion of leprosy patients in the
with definite loss of sensation; (ii) damage to the peripheral community to very low levels, specifically to fewer than 1
nerves, as demonstrated by loss of sensation and mobility to case per 10,000 population. WHO hopes to show that leprosy
hand, feet, or face; or (iii) a positive skin smear even with is not an endless problem, that it is even curable, and that,
treatment that has not been completed. within a foreseeable period and through the use of an
effective treatment regimen, it is possible to reduce the
prevalence of the disease to such a low level that it would no
longer constitute a public health problem.
Disability The leprosy elimination strategy has polarized opinions. It
has it strengths, such as increased commitment by govern-
Difficulty or impossibility to carry out specified activities ments, donors, and health workers to focus on leprosy and
because of primary or secondary impairments. facilitate free drug treatment. New case detection has been
promoted with innovative approaches. Unfortunately, the
terminology of leprosy elimination is confusing and
misleading. Creating a target to eliminate leprosy should
Disability grading
be set only if it is realistic.10
A World Health Organization (WHO) system of grading
of the leprosy-related disabilities involving the eyes, hands,
and feet. It consists of a three-point scale (0, 1, 2). The
highest grade of disability of any of these body sites is used
as an overall indicator of the disability status of a person
with leprosy. Its main use is to assess the disability burden in
the community for decision making, as an indirect indicator
for assessing the performance of early case detection, and
for grading the potential for preventing disabilities in
individual patients.
Drop foot
Entropion Impairment
An inversion of the eyelid margin. Changes in the structure and functioning of certain
parts of the human body caused by a disease. There may
be primary and secondary impairments in leprosy. The
former results directly from the disease, such as nerve
Genetics and leprosy damage or eye damage. The latter is secondarily related
to the initial damage of the disease, such as ulcers
The existence of a strong genetic component in leprosy resulting from unprotected use of insensitive hands or
susceptibility. To date, it is not clear what the exact nature feet12 (Figure 5).
of the genetic factors involved may bethat is, the
identity and number of genes, plus the variants of such
genes involved.
Indeterminate leprosy
HIV-leprosy coinfection
Keratitis
Keratoconjunctivitis sicca
Lagophthalmos
aspect to the patient; therefore, this clinical type of
lepromatous leprosy (LL) is also called lepra bonita
Inability to completely close the eyelids, leading to (pretty leprosy). Lepra bonita was supposed to be unique
persistent exposure of the ocular globe (Figure 6). to Mexico, but a few cases have been diagnosed in Brazil
and India.
Stigma as a negative response to human differences. Loss of eyebrows and eyelashes in leprosy caused by
These may be obvious visible signs or differences in direct M leprae invasion of the hair follicles with subsequent
behavior, or they may be more subtle. If these are related atrophy (Figure 10).
to a health condition, this response is labeled health-
related stigma.13
Miotic pupils
Fig. 9 Nodules in the ear in a lepromatous leprosy patient. Fig. 10 Madarosis in a lepromatous leprosy patient.
Leprosy: A glossary 15
prognosis. It is negative in lepromatous (LL) and borderline not directly associated with an acute reactive episode, may
lepromatous (BL) types. In indeterminate cases, a negative test be present in a considerable proportion of cases. Neural
may indicate that the lesion is differentiating into lepromatous inflammation can excite and sensitize nociceptors. In some
leprosy and may thus be helpful in assessing the direction in cases, there is severe destruction of nerve fibers. The partial
which the disease will progress. regeneration that follows may produce discharges, dimi-
nution of stimulus thresholds, and exaggerated responses
of nociceptors.
Mouse footpad test (Shepard method)
A system to detect drug resistance in leprosy and study the Orbital apex syndrome
viability of M leprae. A suspension of M leprae is inoculated
into the mouse footpad of normal or thymectomized, irradiated Pathologic condition leading to deterioration of tissues
mice. Animals receive a daily food ration with the studied lying in superior orbital fissure (cranial nerve [CN] III, CN
drug, and microscopic examination of a biopsy from the IV, V1 branches of the CN V and CN VI) and in the optic
footpad injected indicates the amount of bacilli after a period of nerve canal (CN II), leading to ptosis, ophthalmoplegia, loss
observation. The mouse footpad technique is labor intensive, of vision, decreased ocular sensations, and secondary corneal
time consuming, and expensive in terms of the costs of animal ulceration and opacities.
purchase and maintenance; however, it is still the gold standard
to study drug resistance and viability of the pathogen.
Participation scale
Multibacillary leprosy (MB) A standardized interview tool for assessing the severity
of rehabilitation needs of people with disability and those
A simplified classification of leprosy for field purposes with other stigmatizing conditions. The instrument is based
indicating cases with high bacillary load for purposes of on the Participation domains of the International Classifi-
MDT treatment. MB patients are those leprosy patients with cation of Functioning, Disability, and Health (ICF-WHO)
more than five skin lesions. and aims to measure client-perceived participation in people
affected by leprosy, allowing collection of participation
data and impact assessment of interventions to improve
Multidrug therapy (MDT) social participation.15
A direct consequence of a lesion or a disease affecting PGL-1 of M leprae represents a specific antigen for leprosy
the somatosensory system. In leprosy, neuropathic pain, that may be of help for the detection of early leprosy.16
16 M. Virmond et al.
Prevalence rate
Ptosis
The number of cases of leprosy in a defined population at
a specific time, usually December 31 of a given year, divided An abnormally low position of the upper eyelid.
by the population in which the cases occur. Point prevalence
rate is the number of current new and preexisting cases at a
specified time.
Relapse
A leprosy patient with clinical presentation limited to Reactions in leprosy associated with the development of
the peripheral nerves. PNL is also called neural leprosy, M leprae antigenic determinants. They are delayed
hypersensitivity reactions and may occur in both pauciba-
cillary leprosy and multibacillary leprosy. In type 1 lepra
reactions, there is a high risk of permanent damage to the
peripheral nerve trunks. If the reaction is mild and there is
no evidence of neuritis (pain, loss of sensation or
function), the reaction should be treated with analgesics,
such as acetylsalicylic acid. If there is nerve involvement,
treatment should include analgesics and corticosteroids
(prednisolone) (Figure 12).
Ridley-Jopling classification
Skin smears
Staphyloma
Tarsorrhaphy
Fig. 12 Type 1 leprosy reaction (reversal reaction).
Surgical approximation of the upper and lower eyelids
with sutures. The primary aim of tarsorrhaphy is protection
of the cornea.
Schwann cells
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