159: Leprosy Flashcards
What is the definition of leprosy?
Leprosy is a chronic granulomatous disease primarily affecting the skin and nerves, caused by the obligate intracellular pathogen Mycobacterium leprae.
What are the primary areas affected by leprosy?
Leprosy primarily involves the skin and peripheral nerves, leading to a range of tissue sequelae including loss of sensation, nerve swelling, and other systemic effects.
What are the key epidemiological indicators of leprosy burden?
The key epidemiological indicators include the new case detection rate by country, the proportion of cases in children, those with multibacillary disease, and those with grade 2 disability, indicating late diagnosis.
What has been the trend in the number of new leprosy cases reported by WHO over the last decade?
The number of new leprosy cases reported by WHO has gradually decreased from 265,661 in 2006 to 210,758 in 2015, although issues of late diagnosis persist.
What are the two stable forms of leprosy identified in clinical features?
The two stable forms of leprosy are: 1. Tuberculoid leprosy 2. Lepromatous leprosy
What is the significance of the new case detection rate in understanding leprosy epidemiology?
The new case detection rate is significant as it reflects the effectiveness of leprosy control measures and indicates the presence of undiagnosed cases, which can inform public health strategies.
What challenges are associated with the diagnosis and treatment of leprosy?
Challenges include the long incubation period, issues with misdiagnosis, and the fact that there is currently no laboratory test to diagnose leprosy, leading to delays in treatment.
How does the classification system by Ridley and Jopling categorize leprosy?
Ridley and Jopling’s classification system categorizes leprosy into five groups based on clinical, histopathologic, and immunologic criteria, reflecting the clinical spectrum of the disease.
What is leprosy caused by?
Mycobacterium leprae.
What tissues does leprosy primarily infect?
Mucous cutaneous tissues and peripheral nerves.
What is the WHO’s definition of a leprosy diagnosis in endemic countries?
Any individual presenting skin lesions with definite sensory loss or positive skin smears may be diagnosed with leprosy.
What trend has been observed in the number of new leprosy cases over the last 10 years according to WHO?
The number of new cases is gradually decreasing.
What percentage of the global leprosy burden is accounted for by India, Brazil, and Indonesia?
More than 80%.
What is the significance of grade 2 disability in leprosy cases?
It indicates late diagnosis.
What is the current status of laboratory tests for diagnosing leprosy?
There is currently no laboratory test to diagnose leprosy.
What are the two stable forms of leprosy?
Tuberculoid leprosy and lepromatous leprosy.
What is the impact of multidrug therapy on leprosy treatment duration?
Treatment was shortened to, at most, 2 years.
What is the main challenge in diagnosing leprosy?
Delay and absence of diagnosis seem to be the main problems.
What are the primary clinical challenges associated with the diagnosis and management of leprosy?
The primary clinical challenges include:
- Long incubation period: Symptoms may take 3-7 years to develop, complicating early diagnosis.
- Misdiagnosis: Many individuals may not be correctly diagnosed due to the subtlety of early symptoms.
- Late diagnosis: High rates of grade 2 disability indicate that many cases are diagnosed too late.
- Lack of laboratory tests: There is currently no definitive laboratory test for leprosy, relying instead on clinical signs and symptoms.
- Stigmatization: Social stigma associated with leprosy can hinder individuals from seeking treatment.
How does the epidemiology of leprosy in India, Brazil, and Indonesia reflect the global burden of the disease?
India, Brazil, and Indonesia account for over 80% of the global leprosy burden. Key points include:
- India has the highest number of leprosy cases worldwide.
- Brazil has the highest new case detection rate among all countries.
- Both countries report high percentages of cases in children and those with grade 2 disability, indicating late diagnosis.
- Despite a decrease in new cases globally, the prevalence remains stable, suggesting ongoing challenges in detection and treatment.
What are the two stable forms of leprosy and how do they differ in terms of clinical presentation?
The two stable forms of leprosy are:
-
Tuberculoid leprosy:
- Characterized by a strong immune response.
- Fewer lesions, often with well-defined borders.
- Typically presents with sensory loss in affected areas.
-
Lepromatous leprosy:
- Characterized by a weak immune response.
- More numerous lesions, often diffuse and poorly defined.
- Can lead to significant nerve damage and disability.
The borderline group exists between these two forms, indicating varying immune responses and clinical presentations.
What implications do the findings regarding the new case detection rate and the proportion of cases in children have for leprosy management strategies?
The findings suggest several implications for leprosy management strategies:
- Focus on early detection: High new case detection rates indicate a need for improved screening and awareness programs, especially in endemic regions.
- Targeting children: The significant proportion of cases in children highlights the necessity for targeted interventions in pediatric populations to prevent transmission and disability.
- Addressing late diagnosis: The stable high rates of grade 2 disability suggest that educational campaigns are needed to reduce stigma and encourage early medical consultation.
- Resource allocation: Countries with high case detection rates may require more resources for treatment and rehabilitation services to manage the long-term effects of the disease.
What is the significance of cell-mediated immunity in the progression of leprosy?
Cell-mediated immunity is crucial in preventing disease progression in leprosy, with more than 90% of people having a natural immunity. It plays a key role in determining the clinical spectrum of leprosy, influencing whether a patient develops indeterminate leprosy, polar tuberculoid leprosy, or polar lepromatous leprosy.
What are the characteristics of polar tuberculoid leprosy?
Polar tuberculoid leprosy is characterized by:
- Better cell-mediated immunity
- Well-defined plaques: Usually a few in one segment of the body, hypochromic and/or erythematous.
- Presence of papules or tubercles: Mainly circinate on the periphery of the lesions.
- No infiltration: Papules or tubercles do not show infiltration.
- Skin dryness and alopecia: Can present both dryness of the skin and alopecia restricted to the territory of the lesions.
What defines polar lepromatous leprosy and its clinical features?
Polar lepromatous leprosy is defined by:
- Complete lack of cell-mediated immunity
- Plentiful nodular lesions: Disseminated throughout the body.
- Diffuse infiltration: Including the ears and face.
- Leonine facies: Marked facial features resembling a lion’s face.
- Extensive areas of dryness: Especially on the legs, with advanced cases leading to madarosis and hair loss.
What are the three borderline forms of leprosy and their characteristics?
The three borderline forms of leprosy are:
Form | Characteristics |
|————————–|—————————————————————————————————-|
| 1. Borderline-tuberculoid | - Clear infiltrative band around the periphery of lesions.
- More diffuse infiltrated outer layer compared to tuberculoid leprosy.
- Restricted features of dryness of the skin and alopecia. |
| 2. Borderline-borderline | - Classical foveolar lesions.
- Immunologically unstable. |
| 3. Borderline-lepromatous | - Decreased cell-mediated immunity, leading to increased bacilli.
- More nodular lesions, often involving the face and ears.
- More diffuse features of dryness of the skin and alopecia. |
A patient with leprosy presents with leonine facies and diffuse skin infiltration. What type of leprosy is this, and what is the immune response?
This is lepromatous leprosy, characterized by a Th2 immune response with high antibody production and defective cell-mediated immunity.
What is the overall genetic resistance toward developing leprosy?
More than 90% of people have a natural immunity, with cell-mediated immunity being most important in preventing disease progression.
What are the initial skin manifestations of leprosy in patients without primary neural leprosy?
They first present with one or a few hypopigmented macules on the skin.
What characterizes polar tuberculoid leprosy?
It is at one end of the spectrum with better cell-mediated immunity, presenting well-defined plaques, usually a few in just one segment of the body.
What is infantile nodular leprosy?
A special self-healing type of tuberculoid leprosy that can be found as a single nodular lesion or as papules or plaques, usually on the face of the child.
What is the characteristic of polar lepromatous leprosy?
It is at the other end of the spectrum with a complete lack of cell-mediated immunity, presenting plentiful nodular lesions disseminated throughout the body.
What is histoid leprosy?
A special type of lepromatous leprosy that has an even higher bacillary load than usual lepromatous leprosy, presenting diffuse shiny nodules and papules.
What are the three borderline forms of leprosy?
They are borderline-tuberculoid, borderline-borderline, and borderline-lepromatous, all of which are immunologically unstable.
What happens to cell-mediated immunity in borderline-lepromatous leprosy?
Effective cell-mediated immunity decreases, allowing a progressive spread and increase in the number of bacilli.
What is the significance of the presence of Th1 and Th2 cytokines in leprosy?
Th1 cytokines are present in the paucibacillary pole (polar tuberculoid leprosy), while Th2 cytokines are present in the multibacillary pole (lepromatous leprosy).
What is the role of acid-fast bacilli and anti-PGL-I IgM in leprosy diagnosis?
They are low or negative in paucibacillary leprosy and increase through the multibacillary pole.
What is the significance of cell-mediated immunity in the progression of leprosy, particularly in indeterminate leprosy?
Cell-mediated immunity plays a crucial role in preventing the progression of leprosy. In indeterminate leprosy, the outcome may depend on the host’s immune response, leading to either a spontaneous cure or progression towards polar or borderline forms of the disease. The effectiveness of this immunity can determine the clinical spectrum of leprosy, influencing whether a patient develops tuberculoid or lepromatous leprosy.
How do the clinical features of polar tuberculoid leprosy differ from those of polar lepromatous leprosy?
The clinical features of polar tuberculoid leprosy include:
- Better cell-mediated immunity
- Well-defined plaques, usually few in number, hypochromic or erythematous, sometimes atrophic
- Presence of papules or tubercles with no infiltration
- Possible dryness of the skin and alopecia restricted to the lesions’ territory.
In contrast, polar lepromatous leprosy is characterized by:
- Complete lack of cell-mediated immunity
- Plentiful nodular lesions disseminated throughout the body
- Associated with diffuse infiltration, including leonine facies and extensive areas of dryness, especially on the legs.
What are the three borderline forms of leprosy and how do they differ in terms of immune response and clinical presentation?
The three borderline forms of leprosy are:
1. Borderline-tuberculoid:
- Clear infiltrative band around the lesions
- More diffuse infiltrated outer layer compared to tuberculoid leprosy
- Restricted features of dryness and alopecia.
-
Borderline-borderline:
- Classical foveolar lesions
- Immunologically unstable with variable features.
-
Borderline-lepromatous:
- Decreased cell-mediated immunity
- Progressive spread and increase in bacilli
- More diffuse features of dryness and alopecia, with evolution to more nodular lesions, often involving the face and ears.
What is the clinical significance of Lucio leprosy and its distinguishing features?
Lucio leprosy is characterized by:
- Main skin manifestation as infiltration
- It poses a diagnostic challenge for less experienced health professionals.
- First described by Lucio and Alvarado in 1852, it is associated with Mycobacterium lepromatosis, a newly identified species.
- It presents with diffuse shiny nodules and papules, and a higher bacillary load than typical lepromatous leprosy, indicating a more severe form of the disease.
What are the key components of a thorough sensitivity evaluation for leprosy lesions?
A thorough sensitivity evaluation for leprosy lesions includes the following components:
- Vasomotor reflex
- Sweating function
- Thermal sensitivity
- Pain sensitivity
- Tactile sensitivity
What is the significance of the Triple Lewis response in leprosy diagnosis?
The Triple Lewis response is significant in leprosy diagnosis as it assesses nerve integrity through the following phases:
- Erythema: Occurs within 5-10 seconds after histamine injection.
- Secondary Erythema: Develops 2 minutes later, indicating capillary dilation, observed only in normal skin.
- Wheal Formation: The last phase involves exudation of liquid to the dermis, resulting in wheal formation in both normal and lesional skin.
The response is incomplete in lesional skin, indicating nerve damage.
How does the Semmes-Weinstein monofilament test assess sensory loss in leprosy patients?
The Semmes-Weinstein monofilament test assesses sensory loss in leprosy patients by:
- Using monofilaments of varying thickness to apply different amounts of pressure to the skin.
- Measuring the patient’s ability to feel light touch, with specific colors indicating different levels of sensation:
Thickness | Color | Range | Sensation |
|———–|——-|——-|———–|
| Thinnest | Green | 0.008 - 0.07 g | Normal skin, mosquito landing |
| Blue | Blue | 0.16 - 0.4 g | Light touch |
| Purple | Purple| 0.6 - 2 g | Protective |
| Thickest | Red | 4 - 300 g |
What is the purpose of the Semmes-Weinstein Monofilaments in leprosy evaluation?
Semmes-Weinstein Monofilaments are used to measure different levels of protective sensation in the skin, indicating nerve damage.
What are the characteristics of tuberculoid leprosy in terms of nerve involvement?
Tuberculoid leprosy may show conspicuous alterations in just one specific peripheral nerve trunk, usually in the same segment as the skin lesion.
What are the noncutaneous findings associated with leprosy?
Noncutaneous findings include palpation of peripheral nerve trunks, loss of sensation, motor dysfunction, and autonomic alterations.
What diagnostic tests would you perform to confirm leprosy?
Perform a sensitivity evaluation, use the Triple Lewis response test, Semmes-Weinstein monofilaments, and conduct a skin biopsy with Fite-Faraco staining.
What does an incomplete Triple Lewis response indicate in leprosy?
It indicates nerve damage in the lesional skin.
What is the basis for the diagnosis of leprosy?
The diagnosis is based on the detection of hypo- or total anesthesia in the lesions, which may be associated with hypohidrosis and alopecia.
What symptoms are commonly associated with leprosy?
Common symptoms include paresthesia, burning, numbness, and tickling sensations.
What is the purpose of the Triple Lewis response test in leprosy diagnosis?
The test assesses the dilation of blood capillaries as a secondary axon reflex erythema, which is dependent on nerve integrity.
How is sweating function assessed in leprosy lesions?
Sweating function is assessed using iodine-starch or alizarin red tests, where exercise may be required to induce sweating.
What are the implications of using iodine-starch or alizarin red tests in evaluating sweating function in leprosy lesions?
These tests evaluate sweating function by assessing the skin’s response to these substances, indicating impaired autonomic nerve function.
What are the key considerations when performing thermal sensitivity tests on leprosy patients?
Key considerations include understanding the test purpose, proper technique, recording responses, and avoiding simultaneous contact.
What are the challenges in diagnosing leprosy early?
Challenges include subtle initial symptoms, delayed presentation, and complexity of nerve involvement.
What are the common clinical signs of primary neural leprosy?
Signs include nerve inflammation or functional deficit without skin lesions, and a positive acid-fast bacilli result eliminates primary neural leprosy.
What endocrine dysfunctions are associated with leprosy?
Endocrine dysfunctions include hypothyroidism, euthyroid sick syndrome, hypogonadism, sterility, and osteoporosis.
What is the impact of leprosy on the eyes?
Loss of corneal sensation can lead to wounds, infections, and blindness, along with hypotrophy of palpebral muscles causing lagophthalmos.
What is the significance of Mycobacterium leprae in leprosy?
It is a noncultivable obligate intracellular pathogen that mainly damages skin and peripheral nerves, leading to a range of skin lesions and peripheral neuropathy.
What is the doubling time of Mycobacterium leprae and its clinical significance?
M. leprae has a doubling time of around 13 days, explaining the long incubation time between infection and clinical disease.
What are the potential complications associated with untreated leprosy?
Untreated leprosy can lead to significant complications in the eyes and limbs, including loss of sensation, infections, and severe deformities.
How does Mycobacterium leprae affect the peripheral nervous system?
It attaches to Schwann cells, leading to colonization, inflammation, nerve damage, and peripheral neuropathy.
What is the significance of genetic resistance to leprosy in the population?
More than 90% of people worldwide have a natural immunity to leprosy, reducing the incidence of the disease.
What is the relationship between leprosy and social stigma?
Leprosy leads to disfigurement and deformity, contributing to the origin of stigma associated with the disease.
What is the role of T helper 1 (Th1) and T helper 2 (Th2) cytokines in the immune response to leprosy?
Th1 cytokines promote a pro-inflammatory response, while Th2 cytokines downregulate the inflammatory response, affecting disease progression.
What are the potential complications of leprosy on the hands and feet?
Complications include loss of sensation leading to wounds, muscle atrophy, and severe deformities.
What are the challenges in diagnosing leprosy after 35 years of multidrug therapy?
The challenge is to diagnose cases early to eliminate disabilities.
What percentage of leprosy patients may present with primary neural leprosy?
4-8% of all leprosy cases; in India, it is about 17%.
What diagnostic methods are used for primary neural leprosy?
Clinical signs, nerve histopathology, electrophysiology, and ultrasonography are used for diagnosis.
How does Mycobacterium leprae interact with the host’s immune system?
It binds to pattern recognition receptors on host cells, triggering an innate immune response.
What are the implications of the long doubling time of Mycobacterium leprae on the clinical management of leprosy?
The long doubling time complicates diagnosis and treatment, as symptoms may not appear for years after infection.
How does the ability of Mycobacterium leprae to invade Schwann cells affect the pathogenesis of leprosy?
It leads to colonization and inflammation within nerves, causing nerve damage and demyelination.
What role do Th1 cytokines play in the immune response to leprosy?
Th1 cytokines promote a pro-inflammatory response that helps control bacterial growth and prevent dissemination.
What role do Th2 cytokines play in the immune response to leprosy?
Th2 cytokines downregulate the inflammatory response, leading to uncontrolled growth of the bacteria and ineffective antibody responses, which can worsen disease symptoms.
What genetic factors have been associated with susceptibility to leprosy?
Certain HLA complex genes and SNP differences in genes such as HLA-DR-DQ, RIPK2, TNFSF15, CCDC122, C13orf31, and NOD2.
What do twin studies indicate about leprosy susceptibility?
Twin studies indicate a high concordance (60%-80%) for monozygotic twins developing leprosy, suggesting a genetic component.
What are the proposed pathways for how M. leprae gains access to Schwann cells?
- Naked nerve filaments in the epidermis. 2. Entrance of M. leprae in the epidermis, then to Schwann cells. 3. Phagocytosis of M. leprae by dermal macrophages, which then invade the perineurium. 4. Access through the blood via intraneural capillaries.
How does M. leprae affect Schwann cells after invasion?
M. leprae can dedifferentiate and reprogram adult Schwann cells to stem cell-like cells, promoting dissemination of infection.
What immune response patterns are observed in tuberculoid versus lepromatous leprosy?
Type of Leprosy | Immune Response | Cytokines Produced |
|—————-|—————-|——————-|
| Tuberculoid | Strong Th1 | IL-2, TNF-α, IFN-γ, IL-12 |
| Lepromatous | Skewed Th2 | IL-4, IL-5, IL-10, high antibody levels |
What immune response is likely involved in controlling bacterial growth in leprosy?
A Th1 cytokine-mediated and pro-inflammatory response.
What is the significance of twin studies conducted in India regarding leprosy?
They showed an overwhelming concordance (60%-80%) for monozygotic twin pairs to develop leprosy.
What socioeconomic factors increase the risk of developing leprosy?
Living in endemic areas, poverty, and poor sanitation.
How does M. leprae interfere with host cell processes after invasion?
By inhibiting endocytic maturation, inducing lipid droplet accumulation, and intensifying glucose uptake.
What immune response is observed in tuberculoid leprosy cases?
A strong Th1 response with cytokine production including IL-2, TNF-α, and IFN-γ.
What immune response is observed in lepromatous leprosy patients?
A skewed Th2 response with high levels of antibody production.
What role does the G domain of α-dystroglycan play in M. leprae infection?
It may be necessary for the adherence of M. leprae to Schwann cells.
What is the effect of 9-O-acetyl GD3 ganglioside on Schwann cells during M. leprae infection?
It is involved in anti-apoptotic signaling and nerve regeneration.
What are the implications of the immune response shift from Th1 to Th2 in leprosy progression?
The shift from Th1 to Th2 cytokine production in leprosy leads to heightened cell-mediated responses, downregulation of inflammation, and ineffective antibody responses.
How do genetic factors influence susceptibility to leprosy?
Genetic factors influencing susceptibility to leprosy include twin studies, genomewide analyses, SNP differences, and household contacts.
What are the proposed routes for M. leprae to access Schwann cells?
Naked nerve filaments, entrance through the epidermis, phagocytosis by macrophages, and through the blood via intraneural capillaries.
What role does the immune response play in the interaction between M. leprae and the host?
The immune response plays a critical role by initiating at various phases of the interaction, influencing disease progression, inducing antibody production, and exhibiting a dichotomy in immune response.
What are the clinical implications of the increased expression of 9-O-acetyl GD3 ganglioside in leprosy?
It is involved in anti-apoptotic signaling, may aid in the survival of infected Schwann cells, and contributes to the maintenance of nerve function despite infection.
What are the main differences in immune response between tuberculoid leprosy and lepromatous leprosy?
Feature | Tuberculoid Leprosy | Lepromatous Leprosy |
|———|——————–|——————–|
| Granuloma | Well-organized | Poorly organized, foamy macrophages |
| Immune Cells | CD4+ T cells | CD8+ T cells |
| Cell-mediated Immunity | Good | Defective |
| Antibody Production | None | High titers of antibodies |
| Bacilli Presence | None in slit-skin smear | Large numbers of bacilli |
How do Toll-like receptors (TLRs) contribute to the immune response in leprosy?
TLR-1 and TLR-2 have higher expression in tuberculoid leprosy, while TLR-4 signaling in macrophages stimulates TNF-α, IL-6, and CXCL10 production.
What is the role of insulin-like growth factor I in lepromatous leprosy patients?
Insulin-like growth factor I decreases macrophage antimicrobial capacity, inhibiting M. leprae killing.
What are the characteristics of ENL (Erythema Nodosum Leprosum) in lepromatous leprosy patients?
ENL is characterized by immune complex deposition, upregulation of Th17, IL-6, IL-1β, and neutrophil influx.
What factors are associated with Type 1 reaction or reversal reaction in leprosy?
Type 1 reaction is caused by a specific increase in cell-mediated immunity against M. leprae, associated with CD4+ T cell infiltration and upregulation of cytokines.
What type of leprosy is characterized by erythematous plaques and alopecia?
This is tuberculoid leprosy, characterized by a strong Th1 cell-mediated immune response.
What histopathological findings would you expect in a patient with lepromatous leprosy?
Expect massive infiltration of foamy macrophages filled with bacilli, few lymphocytes, and defective cell-mediated immunity.
What are the immunologic features of erythema nodosum leprosum (ENL)?
ENL is characterized by immune complex deposition, upregulation of Th17, IL-6, IL-1β, and neutrophil influx.
What type of leprosy is associated with madarosis and hair loss?
This is lepromatous leprosy, with histopathological findings including foamy macrophages filled with bacilli.
What are the key immunological differences between tuberculoid leprosy and lepromatous leprosy?
Feature | Tuberculoid Leprosy | Lepromatous Leprosy |
|———|——————–|——————–|
| Granuloma | Well-organized | Poorly organized, foamy macrophages |
| Immune Response | Good cell-mediated immunity | Defective cell-mediated immunity |
| T Cells | Predominantly CD4+ T cells | Predominantly CD8+ T cells |
| Antibody Production | No antibody production | High titers of antibodies to M. leprae |
| Bacilli | No bacilli in slit-skin smear | Large numbers of bacilli |
How does the immune response in lepromatous leprosy contribute to the disease pathology compared to tuberculoid leprosy?
In lepromatous leprosy, there is a massive infiltration of foamy macrophages filled with bacilli, characterized by defective cell-mediated immunity, leading to a higher bacterial load and more severe disease manifestations.
What role do Toll-like receptors (TLRs) play in the immune response to Mycobacterium leprae in leprosy patients?
TLRs such as TLR-1, TLR-2, and TLR-4 mediate monocyte and dendritic cell activation, stimulating cytokine production.
How does the immune response differ in lepromatous leprosy compared to tuberculoid leprosy?
Lepromatous leprosy has a defective cell-mediated immunity with high titers of antibodies and large numbers of bacilli, while tuberculoid leprosy has good cell-mediated immunity and no bacilli found in slit-skin smear.
What is the significance of IL-10 in the context of leprosy?
IL-10 induces phagocytosis by macrophages and differentiates monocytes into foamy macrophages.
What is the relationship between insulin-like growth factor I and lepromatous leprosy?
Insulin-like growth factor I decreases macrophage antimicrobial capacity and is low in lepromatous leprosy patients.
What is the role of metalloproteinases in leprosy?
Metalloproteinases 2 and 9 are upregulated in Schwann cells, macrophages, and endothelial cells in primary neural leprosy nerves.
What is the impact of Th17 cells in leprosy patients?
Th17 cells are increased in tuberculoid leprosy patients, while IL-10 producing Treg cells are more prevalent in lepromatous leprosy.
How does the immune response in leprosy affect laboratory testing and diagnosis?
The clinical spectrum of leprosy shows a range of pathologic manifestations that depend on the host’s immune response, complicating diagnosis.
What factors contribute to the delay in leprosy diagnosis?
Factors include a reduction in trained leprosy clinicians and laboratory technicians, leading to misdiagnosis or treatment delays.
What is the role of TLR-1 and TLR-2 in tuberculoid leprosy?
TLR-1 and TLR-2 have higher expression in tuberculoid leprosy, facilitating monocyte and dendritic cell activation, which stimulates TNF-α and IL-12 production.
How does TLR-4 signaling affect macrophages?
TLR-4 signaling can stimulate TNF-α, IL-6, and CXCL10 production in macrophages, contributing to the inflammatory response.
What is altered in lepromatous leprosy?
In lepromatous leprosy, TLR signaling is altered, leading to a defective immune response and increased susceptibility to infection.
What is the immune response profile in patients with erythema nodosum leprosum (ENL)?
In patients with erythema nodosum leprosum (ENL), there is an increase in CD4+/CD8+ T cell ratio and high serum levels of TNF-α. ENL is characterized by an influx of neutrophils and upregulation of Th17, IL-6, and IL-1β, indicating a strong inflammatory response. The presence of anti-PGL-1 antibodies and monocyte chemoattractant protein-1 suggests a complex immune interaction that exacerbates the condition. This immune profile highlights the Type III hypersensitivity reaction associated with ENL, leading to significant clinical implications for management.
What laboratory tests can assist in confirming a diagnosis of leprosy?
Laboratory tests that can assist in confirming a diagnosis of leprosy include: 1. Slit-skin smear for bacilli detection. 2. Serological tests for antibodies against M. leprae, such as anti-PGL-1. 3. Skin biopsy to assess histopathological features. 4. PCR tests for M. leprae DNA. 5. Nerve conduction studies to evaluate peripheral nerve involvement.
What is the standard method for laboratory diagnosis of leprosy in a skin biopsy?
The standard for laboratory diagnosis in a skin biopsy is the detection of acid-fast bacilli by the Fite-Faraco modification of the carbol fuchsin technique and characteristic cellular histologic patterns detected by hematoxylin-eosin staining.
How does the Ridley-Jopling classification system categorize leprosy?
The Ridley-Jopling classification system divides leprosy into 5 forms based on the number of acid-fast bacilli and the degree of lymphocytic infiltration and organization.
What are the common histopathologic findings in indeterminate leprosy?
Common findings in indeterminate leprosy include: Nonspecific tissue response, normal epidermis or basal layer of the skin showing melanin reduction, perivascular or perineural, superficial and deep dermal infiltration by few macrophages and lymphocytes, infiltrate surrounds skin appendages, and rarely, there are bacilli in nerves.
What are the histopathologic characteristics of tuberculoid leprosy?
Histopathologic characteristics of tuberculoid leprosy include: Epidermis usually normal, absence of subepidermal clear zone, dermis noncaseating dermal granulomatous process composed chiefly of activated macrophages (epithelioid cells), CD4+ T cells in the center of the epithelioid cells, CD8+ T cells in the mantle surrounding the granuloma, and giant cells of the Langhans type. Granulomas may contact the epidermis and are often arranged around nerves and vessels.
What is the significance of detecting acid-fast bacilli in skin lesions or slit-skin smears?
Detection of acid-fast bacilli in skin lesions or slit-skin smears automatically places the patient in the multibacillary category, which requires 12 months of multidrug therapy.
What are some emerging diagnostic methods for leprosy?
Emerging diagnostic methods for leprosy include: Measuring antibody titers to mycobacterial antigens by rapid lateral flow devices, cell-mediated cytokine release assays, amplification of mycobacterial DNA by PCR, use of metabolomics to detect molecular features specific to M. leprae infection in blood or urine, and molecular detection of the M. leprae-specific repetitive RLEP sequence by PCR.
What does a positive anti-PGL-I titer and RLEP PCR indicate in a child from a hyperendemic area?
This indicates a high risk of developing leprosy. Conduct a thorough clinical examination for early signs of leprosy and monitor the child closely. Consider household contact screening and preventive measures.
What treatment regimen should be initiated for a patient with leprosy and a positive slit-skin smear with a bacillary index of 6+?
Initiate multidrug therapy with dapsone, clofazimine, and rifampicin for up to 24 months.
What does a positive anti-PGL-I titer and symptoms of neuropathy suggest in a leprosy patient?
This suggests active leprosy with nerve involvement. Initiate multidrug therapy and monitor nerve function closely.
What is the bacillary index in leprosy, and how is it established?
The bacillary index is established through bacilloscopy of multiple skin biopsies or slit-skin smears on a logarithmic scale, ranging from 0 to 6+.
What is the significance of detecting acid-fast bacilli in skin lesions or slit-skin smears?
It automatically places the patient in the multibacillary category to receive 12 months of multidrug therapy.
What are some of the tests developed to assist in diagnosing leprosy?
Tests include measuring antibody titers by rapid lateral flow devices, cell-mediated cytokine release assays, amplification of mycobacterial DNA by PCR, and metabolomics.
What is the role of the synthetic disaccharide of PGL-I in leprosy diagnosis?
It is used in lateral flow tests to enhance sensitivity in detecting leprosy patients, even those at the paucibacillary end of the spectrum.
What are the histopathological features of borderline-tuberculoid leprosy?
Features include presence of a subepidermal grenz zone, no well-defined granuloma with organized collections of epithelioid cells, reduction in the frequency of lymphocytes, scarce Langhans cells, and rare acid-fast bacilli.
What characterizes borderline-borderline leprosy histopathology?
Characterized by aggregates of epithelioid cells, scarce dispersed lymphocytes, no Langhans multinucleated giant cells, and increasing numbers of acid-fast bacilli.
What are the key histopathological findings in borderline-lepromatous leprosy?
Findings include presence of a subepidermal grenz zone, aggregates of macrophages, occasional epithelioid cells with abundant cytoplasm, some foamy cells, and a great number of bacilli and some globi.
What are the histopathological features of lepromatous leprosy?
Features include normal to flattened epidermis, subepidermal grenz zone, aggregates and sheets of foamy macrophages admixed with predominantly CD8+ lymphocytes and plasma cells throughout the dermis and into the subcutaneous fat, and huge numbers of acid-fast bacilli and globi found within foamy macrophages (Virchow cells), nerves, arrectores pilorum muscle, follicular epithelium, and sweat glands.
What is the importance of targeting school children in leprosy surveillance?
It helps in early detection of cases by following up on household contacts of diagnosed children.
What is the goal of developing a simple laboratory test for leprosy?
To diagnose leprosy early and facilitate breaking the lines of transmission to eventually reach the goal of leprosy elimination.
What are the key histopathological findings in indeterminate leprosy, and how do they differ from those in tuberculoid leprosy?
Indeterminate leprosy: Nonspecific tissue response, normal epidermis or basal layer of the skin shows melanin reduction, perivascular or perineural infiltration, superficial and deep dermal infiltration by few macrophages and lymphocytes, infiltrate surrounds skin appendages, and rarely, there are bacilli in nerves. Tuberculoid leprosy: Epidermis usually normal, subepidermal clear zone absent, dermis noncaseating dermal granulomatous process composed chiefly of activated macrophages (epithelioid cells), CD4+ T cells in the center of the epithelioid cells, CD8+ T cells in the mantle surrounding the granuloma, and giant cells of the Langhans type.
How does the Ridley-Jopling classification system categorize leprosy, and what is its significance?
The Ridley-Jopling classification system categorizes leprosy into five forms based on the number of acid-fast bacilli present and the degree of lymphocytic infiltration and organization. This classification aids in determining the type of leprosy, which is crucial for accurate diagnosis, appropriate treatment regimen, and understanding the immunological response and potential disease progression.
What are the implications of detecting anti-PGL-I titers and RLEP PCR positivity in individuals living in hyperendemic areas for leprosy?
The detection of anti-PGL-I titers and RLEP PCR positivity in individuals living in hyperendemic areas indicates a high risk of developing leprosy, supports the need for targeted surveillance and early intervention strategies, and highlights the importance of monitoring asymptomatic individuals who may progress to symptomatic disease.
What advancements have been made in laboratory testing for leprosy?
Recent advancements in laboratory testing for leprosy include lateral flow devices for measuring antibody titers to mycobacterial antigens, cytokine release assays to detect immune responses, PCR amplification of mycobacterial DNA, metabolomics to identify molecular features specific to M. leprae infection, and use of LID-1 protein antigen and synthetic disaccharides of PGL-I to enhance sensitivity in detecting leprosy patients.
What are the histopathological characteristics of histoid leprosy?
- Epidermal atrophy
- Subepidermal grenz zone
- Dermis showing sheets of predominantly spindle-shaped cells with nuclear pyknosis and foamy cytoplasm, vacuolated, and arranged in a storiform pattern
- Some polygonal-shaped cells, macrophages, and inflammatory cells are present
- Some may show a pseudocapsule
- Lesion resembles a fibrohistiocytic tumor
- Fit-Foraco stain: large number of acid-fast bacilli, mostly as rafts or globi
- Bacilli can be located in nerves, Schwann cells, eccrine glands, and in the vascular endothelium
- CD68-positive macrophages and spindle cells are present
What type of leprosy is indicated by a patient with a subepidermal grenz zone and foamy macrophages in the dermis?
This is lepromatous leprosy.
What type of leprosy is indicated by a patient with a pseudocapsule and spindle-shaped cells in the dermis?
This is histoid leprosy.
What is a characteristic of borderline-tuberculoid leprosy regarding acid-fast bacilli?
No acid-fast bacilli or they are rare, found more frequently within peripheral nerves, arrectores pilorum muscle, or granulomas.
What defines the presence of a subepidermal grenz zone in borderline-tuberculoid leprosy?
It indicates the presence of a subepidermal grenz zone without well-defined granulomas.
What is observed in borderline-borderline leprosy regarding lymphocytes?
There are scarce dispersed lymphocytes and increasing numbers of acid-fast bacilli.
What are the characteristics of borderline-lepromatous leprosy?
Presence of a subepidermal grenz zone, aggregates of macrophages, and a great number of bacilli and some globi.
What is a key feature of lepromatous leprosy in terms of macrophages?
Huge numbers of acid-fast bacilli and globi are found within foamy macrophages (Virchow cells).
What does histoid leprosy show in the dermis?
Sheets of predominantly spindle-shaped cells with nuclear pyknosis and foamy cytoplasm arranged in a storiform pattern.
What is indicated by the presence of CD68-positive macrophages in histoid leprosy?
It suggests the presence of macrophages and spindle cells in the lesion.
What does the Fite-Faraco stain reveal in histoid leprosy?
A large number of acid-fast bacilli, mostly as rafts or globi.
What histopathological features are characteristic of borderline-tuberculoid leprosy?
- Presence of a subepidermal grenz zone
- No well-defined granuloma with organized collections of epithelioid cells
- Reduction in the frequency of lymphocytes
- Scarce Langerhans cells
- Rare acid-fast bacilli
How does borderline-borderline leprosy differ from borderline-tuberculoid leprosy in terms of histopathological findings?
-
Borderline-borderline leprosy shows:
- Aggregates of epithelioid cells
- Scarce dispersed lymphocytes
- No Langerhans multinucleated giant cells
- Increasing numbers of acid-fast bacilli
What are the key histopathological features of lepromatous leprosy?
- Normal to flattened epidermis
- Presence of a subepidermal grenz zone
- Aggregates and sheets of foamy macrophages
What are foam macrophages?
Aggregates and sheets of foam macrophages with predominantly CD8+ lymphocytes and plasma cells.
What is found within foamy macrophages?
Huge numbers of acid-fast bacilli and globi found within foamy macrophages (Virchow cells), nerves, arrectores pilorum muscle, follicular epithelium, and sweat glands.
What are the histopathological characteristics of histoid leprosy?
Epidermal atrophy.
What is a subepidermal grenz zone?
Presence of a subepidermal grenz zone.
What does the dermis show in histoid leprosy?
Sheets of predominantly spindle-shaped cells with nuclear pyknosis and foamy cytoplasm, arranged in a storiform pattern.
What are the characteristics of cells in histoid leprosy?
Presence of polygonal-shaped cells, macrophages, and inflammatory cells.
What may some lesions show in histoid leprosy?
Some lesions may show a pseudocapsule.
What do histoid leprosy lesions resemble?
Resembles a fibrohistiocytic tumor.
What does the Fit-Foraco stain reveal?
Reveals a large number of acid-fast bacilli, mostly as rafts or globi.
What are the main characteristics of leprosy reversal reaction?
Edema both extracellular as in epithelioid cell granulomas.
What is observed in leprosy reversal reaction?
Increased number of lymphocytes and Langhans giant cells in the infiltrate.
What is seen in leprosy reversal reaction?
Small collections of epithelioid cells.
What is the organization of granulomas in leprosy reversal reaction?
Poorly organized granuloma.
What necrosis is seen in severe cases of reversal reaction?
Fibrinoid necrosis in severe cases of reversal reaction.
Where are bacilli found in leprosy?
Bacilli in macrophages and nerves.
What is the predominant lymphocyte type present in ENL?
The predominant lymphocyte present in ENL is the T-helper cell, whereas T-suppressor cells predominate in lepromatous leprosy.
What are the key areas for evaluating leprosy neuropathy?
Commonly affected sites include:
1. Elbow for the ulnar nerve
2. Carpal tunnel for the median nerve
3. Fibula head for the fibular nerve
4. Tarsal tunnel for the posterior tibial nerve.
What are the main features of Lucio phenomenon or erythema necrotisans?
- Cutaneous or subcutis necrotizing vasculitis
- Fibrinoid necrosis of small and medium-sized vessels
- Necrosis of epidermis and superficial dermis
- Micro-abscess formation
- Angiogenesis
- Endothelial swelling
- Vascular occlusion caused by luminal thrombi
- Deposits of fibrin in small blood vessel walls of the dermis and subcutis
What is the role of high-resolution ultrasound in leprosy neuropathy evaluation?
- Used for peripheral nerve evaluation and established procedure
- Evaluates nerve function impairment in leprosy neuropathy
- Assesses echogenicity, vascularity, and nerve thickening
- Can detect nerve abscesses and entrapment early
- Its utilization for leprosy neuropathy evaluation is still evolving but promising
A patient with leprosy presents with necrotizing vasculitis and fibrinoid necrosis of small vessels. What is the likely diagnosis?
The likely diagnosis is Lucio phenomenon.
A patient with leprosy has developed a mixed inflammatory infiltrate with neutrophils and eosinophils in the dermis. What reaction is this?
This is ENL (Type 2 reaction).
What is a characteristic of leprosy reversal reaction?
Increased number of lymphocytes and Langhans giant cells in the infiltrate.
What is the predominant lymphocyte present in ENL (Erythema Nodosum Leprosum)?
The T-helper cell.
What are the main microscopic features of Lucio phenomenon or erythema necrotisans?
Cutaneous or subcutis necrotizing vasculitis and fibrinoid necrosis of small and medium-sized vessels.
What does leprosy neuropathy manifest as?
Asymmetric focal or multi-focal neuropathy.
How does leprosy neuropathy manifest?
Asymmetric focal or multifocal lesions, mononeuropathy or mononeuritis multiplex.
What is the role of electroneuromyography in leprosy?
It is used for nerve function assessment at diagnosis and for patient follow-up to detect and characterize new lesions.
What is a common evaluation method for leprosy neuropathy?
Nerve palpation and pain evaluation.
What imaging technique is used for evaluating nerve function impairment in leprosy neuropathy?
High-resolution ultrasound.
What are the key areas for evaluating neuropathy in leprosy?
The elbow for the ulnar nerve, carpal tunnel for the median nerve, fibula head for the fibular nerve, and tarsal tunnel for the posterior tibial nerve.
What is a significant concern regarding enlarged nerves in leprosy?
Entrapment of the enlarged nerves may require surgical intervention to decompress the nerve.
What is the significance of nerve abnormalities after multidrug therapy in leprosy?
Worsening of nerve abnormalities was found independent of leprosy classification or presence of reactions.
What are the key histopathological features of leprosy reversal reaction and how do they differ from ENL or Type 2 reaction?
Leprosy Reversal Reaction:
- Edema both extracellular in epithelioid cell granulomas.
- Increased number of lymphocytes and Langhans giant cells in the infiltrate.
- Small collections of epithelioid cells.
- Poorly organized granuloma.
- Fibrinoid necrosis in severe cases.
- Bacilli present in macrophages and nerves.
ENL or Type 2 Reaction:
- Edema and a mixed inflammatory infiltrate in the dermis and subcutis, predominantly neutrophils with eosinophils.
What are the predominant cell types in subcutis?
Predominantly neutrophils with eosinophils and lymphocytes.
What aggregates are found in subcutis?
Aggregates of foamy macrophages, plasma cells, and mast cells.
What conditions are associated with subcutis?
Vasculitis and mixed lobular and septal panniculitis in most cases.
What type of bacteria is found in leprosy?
Bacilli in large numbers, usually granular in appearance.
What are the manifestations of leprosy neuropathy?
Asymmetric focal or multifocal lesions.
What is mononeuropathy in leprosy?
Mononeuropathy or mononeuritis multiplex.
What causes leprosy neuropathy?
Caused by direct damage of nerves by M. leprae or an inflammatory immune response of the host.
What is chronic neuropathy in leprosy?
Chronic neuropathy with acute exacerbations from reversal reaction or ENL.
What are the key evaluation methods for leprosy neuropathy?
- Nerve palpation
- Pain evaluation
- Sensory assessment
- Muscle power measurement
- Autonomic examination
- Electroneuromyography for nerve function assessment and follow-up.
What imaging techniques are utilized in the evaluation of leprosy neuropathy?
High-resolution ultrasound.
What is the use of high-resolution ultrasound in leprosy?
Used for peripheral nerve evaluation and established procedure for nerve function impairment in leprosy neuropathy.
Evaluates echogenicity, vascularity, and nerve thickening.
What can high-resolution ultrasound detect early in leprosy neuropathy?
Can detect nerve abscesses and entrapment early.
What are the clinical implications of high-resolution ultrasound in leprosy?
Its utilization for leprosy neuropathy evaluation is evolving but promising.
What challenges exist in clinical examination of leprosy neuropathy?
Clinical examination of nerve enlargement may be difficult, and robust parameters for follow-up are lacking.
What outcomes are linked to abnormalities in echogenicity in leprosy?
Linked to poor outcomes if echogenicity abnormalities are present or if intraneural Doppler shows areas above normal limits with less than 30.
What does a Doppler show after multidrug therapy?
Areas above normal limits with less than 30% reduction.
What are the primary lesions associated with leprosy?
Macules and patches, popular or nodular lesions, plaques, polymorphous vesiculobullous eruption, and annular lesions.
What are the differential diagnoses for macules and patches?
Hypopigmentation of pityriasis alba, indeterminate leprosy.
What are the differential diagnoses for popular or nodular lesions?
Dermatomyositis, histiocytosis, lymphoma, sarcoidosis, neurofibromatosis, syphilis, leprosy, etc.
What are the differential diagnoses for plaques?
Erythematous plaques, confusion with fungal infections, hypopigmented plaques mimicking other conditions.
What are the differential diagnoses for polymorphous vesiculobullous eruption?
Dermal/epidermal separation, may be seen in ENL.
What are the differential diagnoses for annular lesions?
May mimic leprosy, annular erythemas, sarcoidosis, syphilis, or tinea.
What are the secondary lesions associated with leprosy?
Infacts and ulcers.
What are the implications of infarcts in leprosy?
Seen in Lucio phenomenon and ENL, may mimic septic infarcts.
What causes ulcers in leprosy?
Occur due to vascular occlusion, can be neuropathic or due to venous insufficiency.
What clinical constellations may be confused with leprosy?
Systemic lupus erythematosus-like changes and vasculitis.
What are the implications of systemic lupus erythematosus-like changes?
May present with false positive syphilis tests, antiphospholipid antibodies, and anemia.
What are the implications of vasculitis in leprosy?
Can mimic ENL and Lucio phenomenon, leading to misdiagnosis.
What secondary lesions can occur in leprosy?
Infarcts and ulcers, which may mimic septic infarcts and occur due to vascular occlusion.
What is a common misdiagnosis for leprosy lesions of a nodular character?
They may be misdiagnosed as ‘vasculitis.’
What condition may cause confusion with leprosy plaques?
Erythematous plaques may mimic conditions like fungal infections or sarcoidosis.
What are systemic symptoms associated with leprosy?
Systemic symptoms may include erythema nodosum, false positive syphilis tests, and autoimmune conditions.
What are the primary lesions that can mimic leprosy and how can they be differentiated?
Primary lesions that can mimic leprosy include:
Lesion Type | Description | Differentiation |
|————-|————-|—————-|
| Macules and patches | Hypopigmentation of ptyriasis alba and indeterminate leprosy | Neurological or histological examination may help distinguish them |
| Popular or nodular lesions | May mimic or be mimicked by various conditions | Requires careful clinical evaluation and possibly biopsy |
| Plaques | Erythematous plaques may resemble leprosy | Appearance and histological features can aid in differentiation |
| Polymorphic vesiculobullous eruptions | May occur in ENL | Histological examination is necessary for diagnosis |
| Annular lesions | May mimic leprosy | Clinical features and history are important for differentiation |
What secondary lesions are associated with leprosy and how do they present clinically?
Secondary lesions associated with leprosy include:
Lesion Type | Description | Clinical Presentation |
|————-|————-|———————|
What are infarcts?
Infarcts occur in Lucio phenomenon and ENL. They present as necrotic lesions or septic infarcts.
What are ulcers?
Ulcers result from vascular occlusion. They are seen on plantar surfaces and may be neuropathic or due to venous insufficiency.
What is vasculitis?
Vasculitis may occur in ENL and Lucio phenomenon. Clinically, it resembles other forms of vasculitis, requiring careful diagnosis.
What clinical constellations may be confused with leprosy?
Clinical constellations that may be confused with leprosy include:
Condition | Distinguishing Features |
| - | - |
| Systemic lupus erythematosus | May present with false positive syphilis tests and other systemic symptoms |
| Antiphospholipid syndrome | Associated with specific antibodies and vascular complications |
| Anemia | Can occur in various conditions, including chronic infections |
| Other autoimmune conditions | Require specific serological tests for differentiation |
What are the two types of leprosy reactions?
- Type 1 (Reversal Reaction): Involves mainly peripheral nerves and skin, characterized by a sudden worsening of skin lesions and nerve function impairment without systemic involvement.
- Type 2 (Erythema Nodosum Leprosum - ENL): May present with localized or systemic symptoms, including severe cutaneous reactions and systemic symptoms such as fever and malaise.
What factors increase the risk of reactions and nerve function impairment in leprosy patients?
Factors include:
- Neuropathy present at the time of diagnosis
- Multibacillary leprosy
- Extent of the disease
What is Multibacillary leprosy?
A form of leprosy characterized by multiple lesions and significant nerve involvement.
What are the clinical features of Multibacillary leprosy?
Features include the extent of the disease and the presence of lesions overlying peripheral nerve trunks.
What are reversal reactions in leprosy patients?
Reversal reactions can occur in up to 30% of patients, leading to sudden worsening of skin lesions and nerve function impairment.
What is the required intervention for reversal reactions?
Immediate intervention is required to prevent nerve impairment and permanent disabilities.
What are the systemic symptoms associated with Erythema Nodosum Leprosum (ENL)?
ENL is characterized by systemic symptoms such as fever, edema, and malaise.
What is ENL?
A severe form of leprosy characterized by aggressive vasculitis with immune complex deposition affecting various organs.
How do reversal reactions and ENL occur together?
Reversal reactions and ENL may occur together in some patients, particularly those with borderline forms of leprosy, leading to increased severity of symptoms and complications.
What is the likely diagnosis for a patient undergoing multidrug therapy with bright red, hot, and sensitive skin lesions?
The patient is likely experiencing a reversal reaction. Immediate intervention with corticosteroids, such as prednisolone, is required to prevent nerve impairment and permanent disabilities.
What type of reaction is presented by a patient with systemic symptoms like fever, edema, and malaise?
This is likely an Erythema Nodosum Leprosum (Type 2 reaction). Management includes anti-inflammatory drugs like thalidomide or corticosteroids to control inflammation and systemic symptoms.
What is the likely reaction for a patient with borderline leprosy who develops new nodular lesions and nerve pain?
The patient is likely experiencing a reversal reaction.
Treatment with corticosteroids like prednisolone in a regressive scheme.
What is the likely reaction for a patient with leprosy who has developed erythema and pain in pre-existing lesions?
The likely reaction is a reversal reaction.
Treatment with corticosteroids like prednisolone.
What are leprosy reactions caused by?
Immune response against M. leprae antigens.
What are the two types of leprosy reactions?
Type 1 (reversal reaction) and Type 2 (ENL).
What is acute neuritis in the context of leprosy?
It may be considered a type of reaction.
What factors increase the risk of reactions and nerve function impairment in leprosy patients?
Neuropathy at diagnosis, multibacillary leprosy, extent of disease, and lesions overlying peripheral nerve trunks.
What is the significance of reversal reactions in leprosy?
They can occur in up to 30% of patients and require immediate intervention to prevent nerve impairment and permanent disabilities.
What is ENL in leprosy?
An aggressive vasculitis with immune complex deposition affecting different organs.
What are common symptoms associated with ENL?
Fever, edema, malaise, and various organ involvements such as neuritis and glomerulonephritis.
What is the relationship between bacillary index and ENL episodes?
A high bacillary index and diffuse skin infiltration are important risk factors, with 65% of cases having more than one episode of ENL.
What are the two types of leprosy reactions and how do they differ?
Type 1 (reversal reaction) and Type 2 (ENL) differ in their immune response and clinical manifestations.
What are the two types of leprosy reactions?
- Type 1 (Reversal Reaction) 2. Type 2 (Erythema Nodosum Leprosum - ENL)
What characterizes Type 1 (Reversal Reaction)?
Involves mainly peripheral nerves and skin. Symptoms include sudden worsening of skin lesions and nerve function impairment without systemic involvement.
What characterizes Type 2 (Erythema Nodosum Leprosum - ENL)?
May have localized or systemic symptoms. Associated with vasculitis and can affect multiple organs, leading to symptoms such as fever, edema, and malaise.
What factors increase the risk of reactions and nerve function impairment in leprosy patients?
- Neuropathy present at the time of diagnosis. 2. Multibacillary leprosy. 3. Extent of the disease. 4. Presence of lesions overlying peripheral nerve trunks.
What is the clinical significance of reversal reactions in leprosy patients?
Reversal reactions can occur in up to 30% of patients, particularly in borderline forms of leprosy. They start with a sudden worsening of skin lesions and nerve function impairment, which can lead to nerve impairment and permanent disabilities if not addressed promptly.
What immediate interventions are required for reversal reactions?
Immediate intervention is required to prevent long-term complications.
How does Erythema Nodosum Leprosum (ENL) manifest clinically?
ENL is characterized by aggressive vasculitis with immune complex deposition.
What symptoms may include in Erythema Nodosum Leprosum (ENL)?
Symptoms may include neuritis, panniculitis, glomerulonephritis, arthralgia, epididymitis, orchitis, and eye inflammation.
What are the common skin manifestations of leprosy?
The main skin manifestation is erythema nodosum, which is more palpable than visible. It may be accompanied by erythema polymorphous or severe cutaneous necrotizing vasculitis (Lucio phenomenon).
What systemic symptoms are common in leprosy?
Systemic symptoms such as fever, edema, and malaise are common.
What are important risk factors for leprosy?
A high bacillary index and diffuse skin infiltration are important risk factors, with 65% of cases experiencing more than one episode of ENL.
How do immunologic disorders affect leprosy resolution?
Immunologic disorders, such as HIV and HTLV infections, as well as the use of immunosuppressive drugs or immunobiological medications, can interfere with the resolution of leprosy.
Why is testing leprosy patients for viral infections significant?
All leprosy patients should be tested for HIV, HBV, HCV, and HTLV-1 because viral coinfection decreases the survival of leprosy patients and increases rates of neuritis, nerve function impairment, and leprosy relapses.
What distinguishes reactions from relapses in leprosy?
Reactions are usually acute, with a rapid appearance of new lesions and infiltration of old ones, responding well to treatment with anti-inflammatory drugs. In contrast, relapses are generally slowly progressing, with a resurgence of primary lesions and new lesions, and do not respond to anti-inflammatory drugs.
What are the three groups of medications used for treating leprosy?
The three groups of medications used for treating leprosy are:
- Antibiotics
What are the medications used for treating leprosy?
- Antibiotics: WHO Multidrug Therapy regimen (rifampicin, dapsone, with or without clofazimine)
- Anti-inflammatory or immunosuppressants: control leprosy reactions (prednisone, thalidomide)
- Analgesic drugs: control neuropathic pain.
What are the potential side effects of Dapsone in leprosy treatment?
Potential side effects of Dapsone include:
- Dapsone hypersensitivity syndrome: presents with fever and cutaneous rash, potentially involving internal organs.
- G6PD deficiency: can lead to serious hemolytic events and life-threatening hemolytic anemia.
What is the role of Clofazimine in leprosy treatment?
Clofazimine is a pigment with anti-inflammatory properties and an unknown antibiotic mechanism. It can cause skin hyperpigmentation and skin dryness as side effects. It is administered at a dose of 300 mg once a month and 50 mg per day in multibacillary patients.
What are the adverse effects associated with Rifampicin?
Adverse effects of Rifampicin include:
- Face and neck redness
- Pruritus and cutaneous rash
- Loss of appetite
- Nausea, vomiting
- Diarrhea
- Malaise
- Purpura
- Epistaxis
- Flulike syndrome characterized by fever, myalgia, and headache.
What is the recommended treatment duration for multibacillary and paucibacillary leprosy cases?
For multibacillary cases, the recommended treatment duration is up to 24 months with dapsone, clofazimine, and rifampicin. For paucibacillary patients, the treatment duration is 6 months with dapsone and rifampicin.
What is the likely cause of violet discoloration of the sclera and dyspnea in a patient on multidrug therapy?
The patient likely has G6PD deficiency leading to hemolytic anemia due to dapsone.
Discontinue dapsone and switch to an alternative medication.
What is the diagnosis for a patient presenting with erythema nodosum and systemic symptoms during leprosy treatment?
The diagnosis is ENL (Type 2 reaction).
Recommended medications include thalidomide (if not contraindicated) and corticosteroids.
What is the likely condition for a patient with lepromatous leprosy who has developed severe cutaneous necrotizing vasculitis?
The condition is likely Lucio phenomenon.
Management includes aggressive anti-inflammatory treatment and supportive care.
What is the likely cause of a flu-like syndrome in a patient with leprosy after taking rifampicin?
The flu-like syndrome is an immunologic side effect of rifampicin.
Discontinue the drug and consider alternative antibiotics if necessary.
What additional tests should be performed for a patient with leprosy and a history of HIV, and why?
Test for HBV, HCV, and HTLV-1, as viral coinfections can worsen leprosy outcomes and increase neuritis and nerve function impairment.
What genetic condition should be tested for in a patient with leprosy who has developed severe hemolytic anemia?
Test for G6PD deficiency, as it can lead to hemolytic anemia when using dapsone.
What is the flu-like syndrome?
The flu-like syndrome is an immunologic side effect of rifampicin.
Discontinue the drug and consider alternative antibiotics if necessary.
What additional tests should be performed for a patient with leprosy and a history of HIV?
Tests for HBV, HCV, and HTLV-1 should be performed, as viral coinfections can worsen leprosy outcomes and increase neuritis and nerve function impairment.
What is the main skin manifestation of leprosy?
Erythema nodosum, which may be accompanied by erythema polymorphous or severe cutaneous necrotizing vasculitis (Lucio phenomenon).
What can interfere with the resolution of leprosy?
Immunologic disorders, such as HIV and HTLV infections, immunosuppressive drugs, or immunobiological medications.
What is the significance of testing leprosy patients for HIV, HBV, HCV, and HTLV-1?
All leprosy patients should be tested due to the high percentage of HIV and other viral infections in leprosy patients, which can decrease survival and increase rates of neuritis and relapses.
How can leprosy reactions be distinguished from relapses?
Reactions are usually acute with rapid appearance of new lesions and systemic involvement, while relapses are slow progressing with resurgence of primary lesions and no response to anti-inflammatory drugs.
What are the three groups of medications used for treating leprosy?
1) Antibiotics (WHO Multidrug Therapy), 2) Anti-inflammatory or immunosuppressants, 3) Analgesic drugs.
What is the role of Dapsone in leprosy treatment?
Dapsone is a simple, low-cost, highly effective drug used in a daily dose of 100 mg or 1 to 2 mg/kg, absorbed by the GI tract and eliminated through the kidneys.
How is Dapsone absorbed and eliminated in the body?
Dapsone is absorbed by the gastrointestinal (GI) tract and eliminated through the kidneys.
What is a potential serious side effect of Dapsone?
Dapsone hypersensitivity syndrome, which can present with fever and cutaneous rash, and may involve internal organs, especially the lungs.
What is the dosing regimen for Rifampicin in leprosy treatment?
Rifampicin is administered once per month with supervision, at a dose of 450 mg for children and 600 mg for adults.
What is the significance of the G6PD enzyme in relation to Dapsone?
G6PD deficiency can lead to serious hemolytic events by oxidative stress, affecting a significant number of people worldwide.
What is the recommended treatment duration for multibacillary leprosy cases?
Dapsone, clofazimine, and rifampicin prescribed for up to 24 months.
What are the key differences between reactions and relapses in leprosy?
Reactions in leprosy are typically acute, characterized by a rapid appearance of new lesions and deterioration of neural function, responding well to anti-inflammatory drugs. In contrast, relapses are slow-progressing, often with a resurgence of primary lesions and new lesions, and do not respond to anti-inflammatory drugs.
How can reactions and relapses be distinguished clinically?
A full course of treatment must be confirmed, and histopathology can be useful to differentiate between a reaction and a relapse.
What are the potential complications associated with Dapsone treatment in leprosy patients, particularly regarding G6PD deficiency?
Dapsone can lead to serious hemolytic events in patients with G6PD deficiency, which is common in tropical areas. Complications include:
- Methhemoglobinemia - Clinically detected as a violet color on sclera, lips, and extremities, along with malaise, headache, and dyspnea.
- Severe hemolytic anemia - Life-threatening condition requiring medication change.
What is a life-threatening condition requiring medication change?
Dapsone hypersensitivity syndrome - A rare but potentially fatal event presenting with fever, cutaneous rash, and possible involvement of internal organs, especially the lungs, leading to eosinophilic infiltrates and pneumonia.
How does the use of Clofazimine in leprosy treatment affect patients?
Clofazimine is used in leprosy treatment for its antibiotic and anti-inflammatory properties. Notable effects include:
What is skin hyperpigmentation?
Due to its affinity for fat tissue and macrophage deposits, leading to noticeable pigmentation in lesions.
What is skin dryness?
Contributes to a xerodermic appearance of the skin.
What is the dosage for Clofazimine?
Administered at 300 mg once a month and 50 mg per day in multibacillary patients.
What impact do these side effects have on patients?
These side effects can significantly impact patient quality of life and adherence to treatment.
What are the adverse effects associated with Rifampicin?
Rifampicin, while highly bactericidal, has several adverse effects that can impact patient management, including:
What is face and neck redness?
An adverse effect associated with Rifampicin.
What is pruritus and cutaneous rash?
An adverse effect associated with Rifampicin.
What are the symptoms of loss of appetite, nausea, vomiting?
Adverse effects associated with Rifampicin.
What are diarrhea and malaise?
May require cessation of the drug.
What are purpura and epistaxis?
Adverse effects associated with Rifampicin.
What is flu-like syndrome?
Characterized by fever, myalgia, and headache, which may lead to discontinuation of treatment.
What is required due to these side effects?
These side effects necessitate careful monitoring and may require adjustments in therapy to ensure patient safety and treatment efficacy.
What are the recommended treatments for reversal?
Details not provided in the text.
What are the recommended treatments for reversal reactions in leprosy patients?
Prednisone at doses of 1 to 2 mg/kg/d in a regressive scheme, diminishing 10% to 15% of the dose every 15 days, with a complete cycle lasting up to 3 months. If the clinical situation worsens, it may be necessary to revert to a higher dose for 30 to 45 days before tapering off again.
What are the potential long-term side effects of corticosteroid use in leprosy treatment?
- Glaucoma
- Cataracts
- Moon face
- Striae
- Adrenal gland atrophy
- Osteoporosis
- Infectious diseases such as systemic fungal infections and tuberculosis
- Strongyloides stercoralis hyperinfection
What is the role of counseling in the management of leprosy patients?
Counseling is a key process in leprosy patient management, addressing:
- Nerve damage and its management
- Harm reduction
- Combating stigma associated with leprosy
- Providing support for patients and their families
- Encouraging self-care groups that are inclusive and supportive
What preventive measures are recommended for leprosy patients to avoid progression of damage?
Preventive measures include:
1. Massage and hydration of the skin of hands and feet to avoid fissures and ulcers.
2. Use of special boot soles or customized shoes for feet with loss of sensation.
3. Self-examination for early detection and immediate treatment of trauma.
4. Adduction, abduction, and opposition movements to maintain muscle health.
5. Use of lubricating agents.
What is the significance of contact examination in leprosy prevention?
Contact examination is crucial for leprosy prevention as it should be a mandatory part of leprosy programs in endemic countries. It identifies household contacts who have a 5- to 8-fold higher risk of contracting leprosy, helps in early detection of cases, and raises awareness in the community to diminish stigma and increase detection of early cases.
What preventive measures should be taken for a patient with leprosy who develops lagophthalmos?
Use lubricating eye drops to prevent keratitis. In severe cases, refer the patient for surgical correction to prevent infection and blindness.
What are the treatment options for a patient with leprosy who reports chronic neuropathic pain?
Treatment options include tricyclic antidepressants (e.g., amitriptyline), anticonvulsants (e.g., gabapentin), and corticosteroids for acute episodes. Physical therapy and counseling may also help.
What interventions can help improve functionality for a patient with leprosy who has developed claw hand?
Reconstructive surgery can help recover functional aspects of the hand. Physical therapy and adaptive tools may also improve functionality.
What is the recommended procedure for a patient with leprosy who presents with a nerve abscess?
Perform abscess drainage and send the content to the laboratory for investigation.
What preventive measures should be taken for a patient with leprosy who has developed osteoporosis during corticosteroid treatment?
Preventive measures should be taken to address the risk of osteoporosis, although specific measures are not detailed in the provided text.
What preventive measures should be taken for osteoporotic treatment?
Recommend calcium intake of 1200-1500 mg/day and vitamin D supplements. Monitor bone health regularly.
What alternative treatments can be considered for a patient with leprosy who has a history of dapsone hypersensitivity syndrome?
Consider alternative antibiotics like ofloxacin, minocycline, or clarithromycin.
What surgical intervention can be considered for a patient with leprosy who has developed lagophthalmos?
Surgical correction of lagophthalmos can prevent keratitis, infection, and blindness.
What is the recommended procedure for a patient with leprosy who has developed a nerve abscess?
Perform abscess drainage and send the content to the laboratory for investigation.
What preventive measures should be taken for a patient with leprosy who has developed osteoporosis during corticosteroid treatment?
Recommend calcium intake of 1200-1500 mg/day and vitamin D supplements. Monitor bone health regularly.
What is the recommended treatment for reversal reactions in leprosy?
Prednisone at doses of 1 to 2 mg/kg/day in a regressive scheme, diminishing 10% to 15% of the dose every 15 days.
What must be controlled during the use of corticosteroids in leprosy treatment?
Glucose levels and blood pressure.
What are some long-term side effects of corticosteroid use?
Glaucoma, cataracts, moon face, striae, adrenal gland atrophy, osteoporosis, and systemic fungal infections.
What is the role of thalidomide in treating leprosy?
Thalidomide may be used to treat ENL (erythema nodosum leprosum) with doses of 100 to 400 mg/day, but it is teratogenic and requires pregnancy testing and contraceptive methods.
What is a key preventive technique for leprosy patients to avoid damage?
Massage and hydration of the skin of hands and feet to avoid fissures, ulcers, and fixed claw formation.
Why is self-examination important for leprosy patients?
It is mandatory for early detection and immediate treatment of trauma.
What is the significance of counseling in leprosy management?
Counseling is essential for managing nerve damage and reducing stigma associated with leprosy.
What is the recommended approach for household contacts of leprosy patients?
Household contacts should be examined as they have a 5- to 8-fold higher risk of contracting leprosy.
What is the current recommendation regarding chemoprophylaxis for leprosy contacts?
There is no current official recommendation to use chemoprophylaxis in leprosy contacts due to lack of significant long-term protection.
What is the role of BCG vaccination in leprosy prevention in Brazil?
BCG revaccination is recommended for all household contacts to provide protection against leprosy.
What are the recommended treatments for reversal reactions in leprosy patients, and what monitoring is necessary during treatment?
Reversal reactions can be treated with prednisone at doses of 1 to 2 mg/kg/d in a regressive scheme, reducing the dose by 10% to 15% every 15 days for up to 3 months. Monitoring of glucose levels and blood pressure is necessary during corticosteroid use to manage potential side effects such as glaucoma, cataracts, and osteoporosis.
What are the potential long-term side effects of corticosteroid use in leprosy treatment?
Long-term use of corticosteroids can lead to various side effects, including glaucoma, cataracts, and osteoporosis.
What are the side effects of long-term corticosteroid use in leprosy treatment?
Long-term use of corticosteroids in leprosy treatment may lead to several side effects, including:
1. Glaucoma
2. Cataracts
3. Moon face
4. Striae
5. Adrenal gland atrophy
6. Osteoporosis
7. Infectious diseases such as systemic fungal infections and tuberculosis
8. Strongyloides stercoralis hyperinfection
What precautions are necessary for women of childbearing age using thalidomide in leprosy treatment?
Thalidomide is a teratogenic drug, which means it can cause birth defects. Therefore, it is mandatory for women of childbearing age to undergo pregnancy testing and to use two contraceptive methods before starting therapy with thalidomide to prevent potential harm to a fetus.
What role does counseling play in the management of leprosy patients?
Counseling is a key process in leprosy patient management, focusing on:
- Addressing nerve damage and its management
- Providing harm reduction strategies
- Combating stigma associated with leprosy
- Supporting reintegration into society
- Offering resources for self-care and disability management
What preventive measures are recommended for leprosy patients to avoid progression of damage?
Preventive measures for leprosy patients include:
1. Massage and hydration of the skin of hands and feet to avoid fissures and ulcers.
2. Use of special boot soles or customized shoes for feet with loss of sensation.
3. Self-examination.
What is self-examination for?
Self-examination is for early detection of trauma.
What movements should be performed to maintain muscle health?
Performing adduction, abduction, and opposition movements helps maintain muscle health.
What is the purpose of using lubricating eye drops?
Using lubricating eye drops prevents keratitis in cases of lagophthalmos.
When should a referral to specialized centers be made?
Referral to specialized centers is for complex cases requiring a multiprofessional team.
What is the role of cell-mediated immunity in leprosy?
Cell-mediated immunity is crucial in preventing disease progression in leprosy.
What skin manifestation is commonly seen in patients with primary neural leprosy?
Patients with primary neural leprosy typically present with 1 or a few hypopigmented macules on the skin.
Who is at the highest risk of developing leprosy?
Individuals with close contact to untreated leprosy patients or those with compromised immune systems are at the highest risk.
What type of leprosy is characterized by infiltration as the main skin manifestation?
Lepromatous leprosy is characterized by infiltration as the main skin manifestation.
What is a characteristic of lepromatous leprosy with higher bacillary load?
Lepromatous leprosy with higher bacillary load is associated with more severe symptoms and a greater risk of transmission.
What are the components of multidrug therapy for leprosy?
Multidrug therapy for leprosy typically includes a combination of rifampicin, dapsone, and clofazimine, with alternative drugs available for specific cases.
What is the treatment approach for lepra reactions?
Treatment for lepra reactions often involves corticosteroids to reduce inflammation and manage symptoms effectively.
What is the most important type of immunity in preventing disease progression in leprosy?
Cell-mediated immunity.
What skin manifestation is commonly seen in patients with primary neural leprosy?
Hypopigmented macules.
Who is at the highest risk of developing leprosy?
Individuals with certain risk factors, such as close contact with untreated leprosy patients.
What is a type of leprosy characterized by infiltration as its main skin manifestation?
Lepromatous leprosy.
What is the purpose of multidrug therapy in leprosy treatment?
To effectively treat leprosy and prevent resistance.
What is a common treatment approach for leprosy reactions?
Corticosteroids and other immunosuppressive therapies.
What is the role of cell-mediated immunity in leprosy disease progression?
Cell-mediated immunity is crucial in preventing disease progression in leprosy, as it helps control the infection and limits the severity of the disease.
What initial skin manifestation is common in patients with primary neural leprosy?
Patients with primary neural leprosy typically present with one or a few hypopigmented macules on the skin.
Who is at the highest risk of developing leprosy?
Individuals with close contact to untreated leprosy patients, those with compromised immune systems, and individuals living in endemic areas are at the highest risk of developing leprosy.
What type of leprosy is characterized by infiltration as its main skin manifestation?
Lepromatous leprosy is characterized by infiltration as its main skin manifestation.
What is a characteristic of lepromatous leprosy with a higher bacillary load?
Lepromatous leprosy with a higher bacillary load.
What is a higher bacillary load associated with in lepromatous leprosy?
A higher bacillary load is associated with more severe symptoms and a greater risk of transmission due to the increased number of bacteria present in the skin and other tissues.
What are the components of multidrug therapy for leprosy?
Multidrug therapy for leprosy typically includes a combination of rifampicin, dapsone, and clofazimine, which work synergistically to eliminate the Mycobacterium leprae bacteria.
What is the recommended treatment approach for leprosy reactions?
The treatment for leprosy reactions often includes corticosteroids to reduce inflammation and manage symptoms, along with supportive care and monitoring for complications.
