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India still reports the highest number of leprosy cases in the world and contributed 56.6% (114,451 out of a total 202,185) of global new cases in 2019.1 During year 2018–2019, India reported 120,334 new cases with Annual New Case Detection Rate of 8.69 per 100,000 population. A total of 9227 child cases were recorded, indicating a child case rate of 7.67%. A total of 3666 grade II disability cases were detected among the new leprosy cases during 2018–2019, indicating a grade II disability rate of 2.65 per million population.2 Odisha ranks third in leprosy prevalence in India. It has risen from 1.24 per ten thousand population in 2014 to 1.39 in 2018.3
Children are considered to be the most vulnerable group to infection due to their naïve immunity and close family contacts. As children must have been infected relatively recently, a high child proportion may be a sign of active and recent transmission of the disease and thus an important epidemiological indicator.3
The disability proportion is defined as the percentages of people with WHO disability grade 1 and 2 respectively among new leprosy cases detected during the reporting year and gives an indication of the delay before diagnosis.3 Deformities hinder social, academic and physical development of a child. They are rare and seen in older children with MB disease or those who present with neuritis.4
One of the main targets of the current WHO 2016–2020 strategy is “to reduce transmission of the disease and reduction of grade 2 disability among new child cases”.5 Being in the post elimination era, awareness about the disease has gone down when compared to ancient times. Leprosy health workers were made multipurpose workers with additional responsibilities for HIV and tuberculosis control. Over the next decade, there was reallocation of resources and a gradual decline in funding for leprosy-related programmes. The perceived drop in prevalence of leprosy paved the way for its integration into the general health care services, with a phasing out of vertical leprosy programmes.6 There is a paradoxical delay in diagnosis and treatment, and an increase in the severity of impairment and risk of avoidable permanent tissue and nerve damage in some countries where marked success in treating Hansen’s disease has occurred.7 There are only a few studies describing childhood leprosy with disability.8,9 This prompted us to conduct “a cross-sectional study on clinical presentation and epidemiological aspects of childhood leprosy with disabilities in the post elimination era” with the following objectives:
(1)
To study the socio-demographic and clinical characteristics of the childhood leprosy cases with disability.
(2)
To categorize types and grades of disabilities due to leprosy.
Materials and method
A cross-sectional observational study was conducted in the Department of Dermatology in a tertiary center from October 2018 to June 2020. All recent cases of childhood leprosy (less than or equal to 13 years) with WHO grade 1 and grade 2 disability attending the department of dermatology and willing to take part in the study were included in the study. Institutional ethics committee approval was obtained prior to initiation of study.
Informed consent was taken from patients and parents/caretakers before collecting clinical information and pictures. A complete history of presenting complaints, duration of illness, contact history, immunization, and treatment was taken. Information on various socio-demographic variables like age, sex, Below Poverty Line/Above Poverty Line status, clinical type of leprosy, treatment given (paucibacillary/multibacillary), lepra reactions, frequency and treatment for the same, deformities, side effects to treatment was obtained. The total duration of delay included both patient delay, which is the time period from when patient noticed symptoms and reported to a health care provider and health care provider delay, which is the time period from when the patient visited the health care provider first and confirmed diagnosis.
A complete general examination of the patient was done. The patients with pallor clinically were investigated to rule out anemia. The palms and feet were checked for trophic changes like callosity, fissures, ulcers and deformities like claw hand or foot drop.
Systemic features like fever, joint pain, edema, and lymphadenopathy were also enquired to diagnose lepra reaction and systemic involvement of Hansen’s disease.
Patients were asked about neuropathic pain and graded accordingly. This was evaluated on a scale from 0 to 3 where:
0 - Patient says “no nerve pain” even when asked about it;
1 - Patient complains of nerve pain even when not asked about it;
2 - Patient complains of severe nerve pain and points to the areas of its radiation;
3 - Pain is severe and it interferes with sleep, patient keeps the limb in a position of rest and avoids/restricts its movement.10
A detailed cutaneous examination assessing the site, number, size, morphology, sensation to touch, pain, temperature of skin lesions was done. The peripheral nerves including supratrochlear nerve, supraorbital nerve, infraorbital nerve and mental nerve over face; greater auricular nerve over neck; clavicular nerve, radial nerve, median nerve, radial cutaneous nerve, ulnar nerve over upper limb; common peroneal nerve, sural nerve, anterior tibial nerve and posterior tibial nerve over lower limbs were examined and palpated to assess thickness and tenderness.
Nerve thickness was evaluated and scaled from 0 to 3,
0 - nerve is not thickened;
1 - nerve is thick compared to contralateral nerve;
2 - nerve thickening is rope like;
3 - nerve is thickened and is nodular or beaded.
Nerve tenderness was also evaluated on palpation and scaled from 0 to 3,
0 - palpation is not painful even when asked about it;
1 - palpation is painful only when asked about it;
2 - palpation is painful by wincing during palpation or says so;
3 - pain is so severe that on palpation patient tries to withdraw the limb.10
Impairment of sensation over hands and feet was checked. The touch sensation over the lesion was assessed by using cotton balls. The pain sensation was checked by using pointed end of pin. Hot water (at 40 °C) and cold water (tap water) in test tubes were used to assess the thermal sensation. The corneal reflex was checked using a cotton wisp.
Routine blood investigations including complete blood routine, liver function test and renal function test were done to rule out systemic involvement. Slit Skin Smear examination was performed and stained with Ziehl–Neelson method. They were graded according to Ridley’s logarithmic scale for bacteriological index.
After classification, they were advised for Multi-Drug Therapy (MDT) paucibacillary or multibacillary packs accordingly. The family of pediatric leprosy patients was thoroughly counseled, basic facts about leprosy explained and the need for regular treatment was stressed. The children with deformities were referred to Physical Medicine and Rehabilitation department in the hospital.
The data collected was entered in MS Excel 2010 software. Single value grouping of quantitative variables and descriptive method of statistical analysis was done. The mean, percentage, frequency, range of quantitative and qualitative variables were analyzed. Data analysis, compilation into tables and charts was done using IBM SPSS statistics 22 software.
Results
A total of 18 (32.1%) out of 56 children with leprosy had disability. The child proportion was 10.4% (51 of the total new 490 leprosy patients who visited during the same study period). Male children (83.3%) outnumbered females (17.7%). The age group observed was from 7 years to 13 years. The mean age reported was 11.2 ± 1.38 years. The most common age group affected was 11 to 13 years. The average duration when disability diagnosed was 11.56 ± 9.07 months, with an average delay of 8.6 ± 3.4 months for grade 1 disability and 11.73 ± 9.5 months for grade 2 disability. 14 cases (77.7%) were newly diagnosed. 4 children (22.2%) had taken MDT MB, out of which 2 were continuing MDT at the time of their visit. All children who had started/completed MDT developed disability (11.1%) before start of treatment according to history. There was no further progression of damage after starting MDT in these children. None of them reported adverse drug reactions after starting MDT. They all had clofazimine induced pigmentation over extremities. They reported frequent absenteeism from school due to disease and social stigma from other students or neighbors which had affected their mental health. 13 children (72.2%) belonged to multibacillary category and 5 children (27.3%) were of paucibacillary category. The family of 17 children (94.4%) belonged to Below Poverty Line status. 9 children (50%) had anemia clinically and hemoglobin ranging from 8 g/dl to 10.9 g/dl. 4 children (22.2%) had significant malnutrition, stunting or thinness, with weight for age, height for age and Body Mass Index for age less than or equal to 2 standard deviations below the mean. No patients had ocular involvement.
According to WHO criteria, 3 children (16.6%) had grade 1 disability and 15 children (83.4%) had grade 2 disability. 7 children (38.6%) had known contact with leprosy cases. The source of infection is mostly a close family member (27.7%) or known distant contact (11.1%) who all had multibacillary disease. The father was the most common source of infection (4 cases or 22.2%) (Figure 1). None of the children had a second dose of BCG or rifampicin prophylaxis even if leprosy cases were reported in the family earlier.
Figure 1.
Pie chart on contact history.
The types of disability and their distribution are detailed in Table 1. 15 children (83.4%) had multiple skin lesions. A majority of them (50%) had 2–5 lesions. 2 children (11.1%) had pure neuritic leprosy and they presented with no skin lesions. The most common clinical type of leprosy reported was borderline tuberculoid leprosy. 7 children (70%) of borderline tuberculoid type belonged to multibacillary type, 2 children (20%) had associated type 1 lepra reaction. Most of the borderline tuberculoid type had multiple skin lesions ranging from 5 to 9 lesions and 9 children (90%) had grade 2 disability of visible small muscle wasting of hands and feet (Figures 2–6). The relationship between clinical spectrum of leprosy and grades of disability is detailed in Table 2. All the patients who presented with disability had involvement of peripheral nerves. The relationship between grades of disability and grades of neural pain and nerve tenderness is described in Table 3. All patients with disability had peripheral nerve thickening. The most commonly involved peripheral nerve was the ulnar nerve. 13 children (72.2%) had grade 1 thickening while 5 children (27.7%) had grade 2 thickening of peripheral nerves. Incidence of lepra reaction and slit skin smear positivity was higher in children with disability than in cases without disability. 4 children (22.2%) with disability had associated type 1 reaction while 1 child (5.6%) had associated type 2 reaction at the time of their visit. When slit skin smear was taken and stained, it was found that 7 children (38.8%) tested positive and 11 children (61.2%) tested negative for acid fast bacilli.
Figure 2.
Pure neuritic type leprosy with partial ulnar claw hand.
Figure 3.
Visible muscle wasting of both palms.
Figure 4.
Borderline tuberculoid leprosy lesion on middle finger with visible muscle wasting.
Figure 5.
Xerosis with callosity over medial and lateral malleolus of left foot.
Figure 6.
Trophic ulcers with loss of nail of right index finger with visible muscle wasting of palms.
Table 1
Types of disabilities
| Type of disability | Grade 1 disability | Grade 2 disability | Total |
|---|---|---|---|
| Muscle wasting | 0 | 15 | 15 (26.7%) |
| Glove and stocking anesthesia | 3 | 6 | 9 (16.07%) |
| Difficulty of movement of joints | 0 | 4 | 4 (7.1%) |
| Claw hand | 0 | 2 | 2 (3.5%) |
| Trophic ulcers | 0 | 2 | 2 (3.5%) |
| Other trophic changes | 0 | 2 | 2 (3.5%) |
| Foot drop | 0 | 1 | 1 (1.78%) |
| Sausage shaped digits | 0 | 1 | 1 (1.78%) |
Table 2
Relationship of disabilities and clinical spectrum of leprosy
| Grade of disability | TT | BT | BB | BL | LL | PNL | IND |
|---|---|---|---|---|---|---|---|
| Grade 1 | 0 | 1 | 0 | 0 | 1 | 1 | 0 |
| Grade 2 | 0 | 9 | 1 | 3 | 1 | 1 | 0 |
| Total | 0 | 10 | 1 | 3 | 2 | 2 | 0 |
| Percentage | (55.5%) | (5.5%) | (16.7%) | (11.1%) | (11.1%) |
Table 3
Disability and nerve involvement
| Grade | Neural pain | Nerve tenderness | ||||
|---|---|---|---|---|---|---|
| Disability | Grade 1 | Grade 2 | Total | Grade 1 | Grade 2 | Total |
| Grade 0 | 2 | 7 | 9 (50%) | 1 | 4 | 5 (27.7%) |
| Grade 1 | 0 | 4 | 4 (22.2%) | 1 | 4 | 5 (27.7%) |
| Grade 2 | 1 | 4 | 5 (27.7%) | 1 | 5 | 6 (33.3%) |
| Grade 3 | 0 | 0 | 0 | 0 | 2 | 2 (11.1%) |
Discussion
The proportion of new leprosy cases with grade 2 deformity, as suggested by the WHO, is an indicator to monitor disease control actions since it is less susceptible to operational factors such as detection delay when compared with leprosy prevalence.11
It is worth mentioning that 15 children (83.4%) had visible deformity (grade 2), while 3 children (16.6%) had only anesthesia without deformity (grade 1). Similarly, in a study by Ghunawat et al. which reported 113 childhood leprosy cases, proportionately increased number of cases with grade 2 deformity (75%) were reported than with grade 1 deformity (25%).12 The increased proportion of grade 2 disability indicates the lag in diagnosis of disease in an earlier stage even in the post elimination era, due to ignorance.13
The ratio of male and female cases reported was 4.7:1. This could be due to female children having less access in seeking medical treatment compared to male children and also due to greater mobility and increased opportunities for contact in male children. The increased incidence in higher age groups may be due to relatively long incubation period of leprosy, failure to report in the early stages of the disease, or delayed diagnosis of lesions in children and difficulty in assessing the sensory loss in younger children.
In our study, 13 children (72.2%) belonged to multibacillary category and 5 children (27.3%) belonged to paucibacillary category. It is similar to study by Ghunawat, which reported that most of the cases presenting with disability belonged to multibacillary spectrum (71.4% in both Grades 1 and 2 disability).12 Although children presented with features similar to adults, rare variants like histoid leprosy, lazarine leprosy were not reported.
The usual mode of entry of lepra bacilli into the body is through respiratory tract in the form of droplets. Hence, children usually get infection from nasal secretions of close family contacts. Multibacillary cases have high bacterial load and prevalence rate was still higher where family contact had either the lepromatous disease or multiple lesions. Hence childhood leprosy may provide an opportunity to detect the index case, usually within the family.
In the present study, the most common disability observed was visible muscle wasting (15 children or 26.7%) followed by glove and stocking anesthesia (9 children or 16.07%). In contrast, according to study on childhood leprosy cases in Western Odisha by Pradhan et al., visible deformities were found in 9.3% of children, with trophic ulcer (42.3%) being the most common followed by claw hand (34.6%).8
All children with disability had single or multiple thickened peripheral nerves involved. In our study, the most common clinical type of leprosy presented was borderline tuberculoid type with multiple skin lesions and belonged to multibacillary type. The most common peripheral nerve involved was ulnar nerve, followed by common peroneal nerve, similar to studies by Ghunawat et al. and Pradhan et al.8,12
Table 4
Comparison of present study with previous published data
| Studies | % of childhood leprosy cases with disability | Mean age/ range in years | M:F | Most commonly observed disability (% affected) | Most common clinical type | Most common number of skin patches | Most common peripheral nerve involved | % of type 1 reaction | % of type 2 reaction | Slit skin smear positivity |
|---|---|---|---|---|---|---|---|---|---|---|
| Present study | 32.1% | 11.2 | 5:1 | Visible muscle wasting (26.7%) | BT | 2–5 | Ulnar nerve | 22.2% | 5.6% | 38.8% |
| Pradhan et al.8 | 9.3 % (Grade 2) | 13.43 | 1.95:1 | Ulcer (42.3%) | BT | NM | Ulnar nerve | 11.3% | 7.9% | NM |
| Babu et al.9 | 11.2% | 10.22 | 1.04:1 | Foot drop/partial claw hand (4.4%) | TT | 1 | NM | 4.4% | 2.2% | 8.9% |
| Chaitra et al.14 | 13.89% | 11–14 years | 1.25:1 | Foot drop (2.78%) | TT | 1 | NM | 2.7% | 2.7% | 8.33% |
| Ghunawat et al.12 | 7.6% | 11–15 years | 2.5:1 | Hand deformity (64.3%) | BT | ≤ 5 | Ulnar nerve | 12.3% | 2.6% | 7.1% |
| Balai et al.15 | 24% | 10–14 years | 2.2:1 | Ulnar claw hand and trophic changes (12.5%) | BT | NM | NM | NM | 9.4% | NM |
*NM- not mentioned
The incidence of lepra reaction (27.8%) and skin smear positivity (38.8%) was relatively high in the present study, when compared to other studies on childhood leprosy cases.9,14 Comparison of present study with previous published studies on childhood leprosy is summarized in Table 4.
The major limitation of the study is that it is a single center, hospital-based study which included a small sample population reporting to our dermatology department. It reflects only the tip of the iceberg of the disease burden in our communities. Hence similar studies need to be conducted at multiple centers for more accurate interpretation. The cross-sectional study design limited the advantage of long term observation of treatment response after initiation of MDT.
Conclusion
A majority of the cases presenting to us were in school-going and preadolescent age group, with higher male: female ratio and a delay in treatment seeking behavior, indicating possible stigma and lack of disease awareness even in a high endemic area. This is further supported by the fact that the most common disability in our study was visible muscle wasting.
The increased incidence and prevalence of grade 2 visible deformity reflects a gap in the system in early case detection at the field level and referral services. Preventive strategies to decrease the prevalence of childhood leprosy and disability include:
(1)
Strengthening patient and community awareness on leprosy including frontline workers and community volunteers for early detection of cases.
(2)
Promoting early case detection through active case-finding (active school surveys/ campaigns) in areas of higher endemicity, effective contact tracing including immediate and annual examinations of household contacts and ensuring proper treatment regimen at the earliest.
(3)
Administration of post-exposure prophylaxis with rifampicin to close contacts of all newly diagnosed cases.
References
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