INTRODUCTION Hansens diseases (leprosy) is a chronic, infectious disease caused by M. leprae.

It affects mainly the skin and the peripheral nerves. Leprosy has been called the great imitator, the clinical presentations are so many because of the combinations of many different primary skin lesions with or without signs of inflammation, sequelae of these lesions and signs and sequelae of peripheral nerve damage.1 The first known written mention of leprosy is dated 600 BC. Leprosy was recognized in the ancient civilizations of China, Egypt and India. All countries of the South-East Asia Region were known to be endemic for leprosy. Throughout history, the afflicted have often been ostracized by their communities and families. This situation has changed since leprosy is now completely curable and there is greater awareness about the disease.2 During 1990s, the prevalence of leprosy fell by 90% because patient completing a course of multi drug therapy have been considered to be cured, but the incidence of the disease, which is varies in direct proportion to case-finding efforts, has yet to be reduced convincingly.3 The number of new cases of leprosy detected worldwide and the prevalence during 2010 as reported by 130 countries; globally, 228 474 new cases were detected. The worldwide registered prevalence at the beginning of 2010 was 192 246 cases. New cases detected during 2010 in 17 countries that reported 1000 new cases. These 17 countries accounted for 95% of the new cases detected worldwide during 2010. The proportion of cases with MB leprosy among new cases ranged in the African Region from 61.72% in the Democratic Republic of the Congo to 99.21% in Kenya; in the Region of the Americas, from 40.88% in Brazil to 83.06% in Cuba; in the South-East Asia Region from 42.33% in Bangladesh to 80.96% in Indonesia; in the Eastern Mediterranean Region, from 61.95% in Yemen to 88.38% in Egypt; and in the Western Pacific Region, the proportions ranged from 29.67% in Kiribati to 93.92% in the Philippines. Hansen disease persists in the United States; 133 cases of HD were reported to the National Hansens Disease Registry in 2002. Most of these cases were found in immigrants.4,5 In this case report, We review the recent literature on Hansens disease. We searched published literatures through Internet, and extracted data through direct review of the literatures. Current epidemiology, classification systems, inclusion of the disease in the differential and diagnosis, and therapy are included. Herein we present a case of leprosy. CASE REPORT Patient Identity

Name Sex Registration # Age Race Address Hospital admission date Examination date Anamnese The Chief Complaint:

: Mr. J : Male : 0-90-26-15 : 17 years old : Aceh : Krueng Doe, Peusangan District Bireuen : August 9th 2012 : August 9th 2012

Patient have lesions consisted of some nodules beetwen 2-3 cm, white and black colours over face, ears, upper and lower extrimities and red nodules on the abdomen and backs that has been occured 2 months before.

Additional Complaint:

Swelling over the face and legs that has been occured about 2 months before admission on hospital.

History of the present illness: A 17-year-old boy presented to our teaching hospital with complaints lesions consisted of some nodules beetwen 2-3 cm, white and black colours over face, ears, upper and lower extrimities and red nodules on the abdomen and backs that has been occured 2 months before admission on the RSUDZA hospital. His mother told that when patient was 14 years old, he had tingling and there was no sensation on his hands and legs. Patient then seek medical advice and treatment on his district hospital, diagnosed with Hansens disease, and given multidrugs therapy, but after a year, patient feels that there was no significant advantage on reducing his complaints with therapy given, and has decided to stop taken medication. One year later patient has started to feel the same complaints and seek medical and treatment again, and treated with the same therapy. In the last 4 months, the patient complaints become worst and he referred to RSUDZA hospital. We also found swelling over the face and legs that has been occured about 2 months before admission on hospital.

History of the past illness Family history of diseases Social history Medical history

: : : :

Leprosy (type not known) Allergy - Denied Hansens Disease - Denied High School Student Dapsone, Clofazimine, Rifampisin, Methylprednisolone,

Ofloxacin, Omeprazole, and other topical corticosteroids. Physical Examination a. Vital Sign General condition Cognition BP HR RR Temperature
b. General state

: Good : Compos mentis : 120/80 mmHg : 90 beats/minute : 18 beats/minute : 36,0 C : Swelling over the face and legs

c. Another abnormal findings:

Medarosis (+) Claw hand (+) Dropped foot (+)

Status of Dermatology Location a/r fascialis, auricularis dextra, abdomen anterior et posterior, brachii et anteebrachi dextra et sinistra. Significant findings: hiperpigmentation and hipopigmentation macules, not well-defined with irregular margination, multiple numularic and simmetryc; macula erythematous, well-defined with irregular margination, multiple policyclic; crusts also noted mainly in the face, upper and lower extrimities; squama covered upper and lower extrimities.

Differential Diagnosis
1. Leprosy type LL with ENL type reactions

2. Pityriasis Alba 3. Psoriasis 4. SLE 5. Mycosis fungoides Supporting Test 1. Sensibility test
-

Ears : hipoestesia (-) Face: hipoestesia (-)

Abdomen: (-) Backs: (-) Extremities: lower and upper (+), but not all lesions Auricular nerve enlargement : (-) Ulnar nerve enlargement Tibial nerve enlargement : (+) : (+)

2. Peripheral nerves enlargement

3. Skin biopsy (slit-skin smear biopsy)

BI: +2 MI: 65% Further Recommended Test a. Complete blood count b. Lepromin skin test
c. Other test if available: PCR , serologic assays, and molecular probes.

Diagnosis Leprosy type LL with ENL type reactions Management Medication Systemic
1. Methylprednisolone tab 8 mg once a day at morning. 2. Clofazimine tab 100 mg three times a day

3. Ofloxacin tab 200 mg once a day. 4. Omeprazole 20 mg once a day. 5. Vitamin B1 once a day. Topical
1. Bethamethason valerate cr 0.1% applied to whole lesions for the body (night). 2. Hydrocortisone cr 0,5% applied to whole lesions for the face (night).

Prognosis Dubia ad bonam

DISCUSSION Leprosy as we known by other name, Hansen Disease (HD); is a chronic infection and inflammation disease caused by a bacillus, Mycobacterium leprae (M. leprae). M. leprae multiplies very slowly and the incubation period of the disease is considered to be about five years. Leprosy is transmitted by air through droplets from the nose and mouth, during close and frequent contacts with untreated cases. Leprosy is one of the least infectious diseases, because over 99% of the population has adequate natural immunity; over 85% of the clinical cases are non-infectious, and an infectious case is rendered non-infectious within one week, most often after the very first dose of treatment.
2,3

M. leprae is an obligate intracellular acidfast bacillus;

reproduces maximally at 27C 30C. Organism cannot be cultured in vitro. Infects skin and cutaneous nerves (Schwann cell basal lamina). In untreated patients, only 1% of organisms are viable. Grows best in cooler tissues (skin, peripheral nerves, anterior chamber of eye, upper respiratory tract, testes), sparing warmer areas of the skin (axilla, groin, scalp, and mid-line of back).6 In this case, we have established the diagnosis based on history taking, physical diagnostics, significant dermatology findings and other dermatology examinations to support our suspicion that patient may have leprosy. In history we found that patient have some lesions consisted of some nodules beetwen 2-3 cm, white and black colours over face, ears, upper and lower extrimities and red nodules on the abdomen and backs that has been occured 2 months before admission on the RSUDZA hospital. His mother told that when patient was 14 years old, he had tingling and there was no sensation on his hands and legs. Patient then seek medical advice and treatment on his district hospital, diagnosed with Hansens disease, and given multidrugs therapy, but after a year, patient feels that there was no significant advantage on reducing his complaints with therapy given, and has decided to stop taken medication. One year later patient has started to feel the same complaints and seek medical and treatment again, and treated with the same therapy. In the last 4 months, the patient complaints become worst and he referred to RSUDZA hospital. From physical examination we found that patient swelling over the face and legs that has been occured about 2 months before admission on hospital, we also noted that patient have some abnormal findings like medarosis, claw hand and dropped foot. On dermatological status we found significant findings hiperpigmentation and hipopigmentation macules, not well-defined with irregular margination, multiple numularic and simmetryc; macula erythematous, well-defined with irregular margination, multiple policyclic; at regio

fascialis, auricularis dextra, abdomen anterior et posterior, brachii et anteebrachi dextra et sinistra. crusts also noted mainly in the face, upper and lower extrimities; squama covered upper and lower extrimities. Based on all findings above, we established that patient have leprosy type LL with ENL type reactions as ours primary diagnosis. According to the World Health Organization, a patient with Hansen Disease or leprosy is defined as having one or more of the following features, and who has yet to complete a full course of treatment: 1) hypopigmented or reddish skin lesion(s) with definite loss of sensation, 2) involvement of the peripheral nerves, as demonstrated by definite thickening with loss of sensation, 3) skin smear positive for acid-fast bacilli. The World Health Organization classification divides cases into 3 types: paucibacillary single-lesion (1 lesion), paucibacillary (25 lesions), and multibacillary (more than 5 lesions).7 The Ridley Jopling classification uses clinical, bacteriologic, histologic, and pathologic criteria to divide HD into 5 categories ranging from tuberculoid tuberculoid to lepromatous (6 categories when indeterminate is also included).8 Reactional States is immunologically mediated inflammatory states, occurring spontaneously or after initiation of therapy. Lepra Type 1 Reactions; downgrading and reversal reactions. Occur in borderline disease. Skin lesions become acutely inflamed, associated with edema and pain; may ulcerate. Edema most severe on face, hands, and feet. Lepra Type 2 Reactions (ENL); Occur in 50% of LL. 90% of cases occur after initiation of therapy. Present as painful red skin nodules arising superficially and deeply, in contrast to true erythema nodosum; lesions form abscesses or ulcerate. Lesions occur most commonly on face and extensor limbs. Lucio Reaction; occurs only in patients from Mexico/Caribbean with diffuse LL. Presents as irregularly shaped erythematous plaques; lesions may resolve spontaneously or undergo necrosis with ulceration.6

Figure 1.0 The Ridley and Jopling classification of leprosy

Source: D L Leiker, E Nunzi, Leprosy in the Light Skin. An illustrated manual, page 36. O.C.S.I. (1984), Bologna, Italy. Credits for this slide: The ILA Atlas of leprosy (2009).

Table 1.0 The Classification of Hansen Disease5

*I indicates indeterminate; TT, tuberculoid tuberculoid; BT, borderline tuberculoid; BB, borderline borderline; BL, borderline lepromatous; LL, lepromatous; PBSL, paucibacillary single-lesion; PB, paucibacillary; and MB, multibacillary.

We also did the test for leprosy based on that 3 cardinal signs of leprosy and we found that patient have some loss of skin sensation at lower and upper extrimities skin but not at all lesions. We also examine skin sensation at lesions on ears, face, backs and abdomen but it all came to negative results. The patient have also ulnar and tibial nerve enlargement on palpation. The diagnosis of leprosy is based on the 3 cardinal signs of the disease, which are: skin patch with loss of sensation; enlarged peripheral nerve; and positive slit-skin smear. It is indeed a protean disease with innumerable possible clinical frames. The skin may show macules, plaques, papules, nodules, diffuse infiltration, secondary lesions like burns, blisters, fissures, ulcers and scars. The flat lesions, macules and plaques, may be present with extreme variability in colour (also depending on the basic skin colour), size, edge, texture of the surface, location, distribution and number. Papules, nodules and the secondary lesions may also appear in a wide range of modalities. Moreover the patient may show damage to the eyes, face, nose, hands, feet, testes and other organs and tissues. The damage, again, may vary a lot. It may be relatively

minimal and, passing through all degrees, it may become very serious. At one end of the clinical spectrum a leprosy patient may present only one or a few hypopigmented patches on the skin and nothing else. On the other hand, in the worst scenario, a patient may be blind, with gross destruction of both hands and feet and loss of sensation over most of his skin. The patient is present, within his anaesthetic skin but, almost isolated from the rest of the world. Between the two extremes innumerable combinations are possible.1,3 On slit-skin biopsy, we found that bacterial index (BI) at +2 and morphologic index at 65%. As the literatures say that tests for HD include serologic assays, slit-skin smears,
5

biopsies, probes, polymerase chain reaction (PCR), and the lepromin test.

Slit-skin smears

provide both bacteriologic indices (ie, measurement of the bacterial load) as well as morphologic indices, which signify viability. The inability to detect bacilli places a case in the paucibacillary categories (indeterminate and tuberculoid tuberculoid). The demonstration of bacilli defines it as multibacillary (borderline tuberculoid, borderline borderline, and lepromatous).5 Unfortunately, 70% of patients have negative smears; by definition, paucibacillary HD lacks evidence of bacilli.9 In endemic areas, a diagnosis may be made without biopsy, in part because the correlation between the clinical diagnosis and histopathology is low.5 Serologic assays for M leprae can detect phenolic glycolipid 1, as well as lipoarabinomannan. Phenolic glycolipid 1 is believed to be specific for M leprae, but lipoarabinomannan is shared by other mycobacteria. The antiphenolic glycolipid 1 Ab titer correlates with bacterial load, being higher in lepromatous than tuberculoid cases. It can be useful as titers correlate with the bacterial index following treatment.5 The lepromin test gauges the patients cell-mediated immune response to M leprae. The result is negative in lepromatous HD and positive in tuberculoid tuberculoid HD. Because the lepromin test is negative in lepromatous HD, it cannot be used to diagnose or confirm HD reliably, but it can assist in classification of recognized cases.5 The patient then treated with both combine systemic and topical medication. We gave methylprednisolone tablet 8 mg once a day at morning, clofazimine tab 100 mg three times a day, ofloxacin tablet 200 mg once a day, omeprazole 20 mg once a day and vitamin B1 once a day. For topical lines we also gave bethamethason valerate cr 0.1% applied to whole lesions for the body at night and hydrocortisone cr 0,5% applied to whole lesions for the face at night. Making the initial diagnosis of HD can be a challenge, and so too may be the treatment: HD requires years of treatment and even more years of follow-up, complicated by reactional states. Hansen disease was previously treated with monotherapy, such as dapsone or rifampin. Monotherapy, however, led to drug resistance.10 Multidrug therapy was introduced by the

World Health Organization in 1982 and includes use of dapsone, rifampin, and clofazimine.5 Therapy of reactions is based type of the reactions. Lepra Type 1 Reactions; treated with prednisone, 4060 mg/d; the dosage is gradually reduced over a 2- to 3-month period. Lepra Type 2 Reactions (ENL); treated with prednisone, 4060 mg/d, tapered fairly rapidly; thalidomide for recurrent ENL, 100300 mg/d. Lucio Reaction; prednisone is not very effective. Since there is no other alternative, prednisone, 4060 mg/d, tapered fairly rapidly.6 In general principles of management patient with leprosy include 6 steps:6
1. Eradicate infection with antilepromatous therapy.

2. Prevent and treat reactions. 3. Reduce the risk of nerve damage.

4. Educate patient to deal with neuropathy and anesthesia.

5. Treat complications of nerve damage.

6. Rehabilitate patient into society.

Figure 2.0 Multi Drug Therapy for Leprosy

Source: http://www.who.int/lep/mdt/en/ and http://www.who.int/lep/mdt/clofazimine/en/index.html.

REFERENCES

1. 2.

Noto, S. Schreuder, PAM. Naafs, B. (2011). The Diagnosis of Leprosy. LML Distance World Health Organization. (2006). Leprosy: Fact Sheet. available at

Learning Guide http://www.searo.who.int/en/Section10/Section20/Section2293.htm. WHO Regional Office for South-East Asia. 3. 4. 5. Wolff, Klaus. et all (2008). Fitzpatrick's Dermatology In General Medicine: Cutaneous World Health Organization. (2011). Weekly Epidemiological record; 36, 2011, 86, Anderson, H. Stryjewska, Barbara. L, Boyanton, Jr, Bobby. R, Schwartz, Mary. Reactions to Drugs - 7th edn; 186: 1786. McGraw-Hill. 389400. available at http://www.who.int/wer. WHO Geneva. (2007). Hansen Disease in the United States in the 21st Century: A Review of the Literature. Arch Pathol Lab Med; 131:982986. Department of Pathology, The Methodist Hospital. 6. 7. 8. 9. 10. Wolff, Klaus. Jonhson, R, Allen. (2009). Fitzpatrick's Colour Atlas and Synopsis of World Health Organization. (1997). 7th WHO Expert Committee on Leprosy. available Jacobson, R, R. Krahenbuhl, J, L. (1999). Leprosy. Lancet; 353:655660. Ustianowski, A, P. Lockwood, D, N. (2003). Leprosy: Current Diagnostic and Katoch, V, M. (2002). Advances in the diagnosis and treatment of leprosy. Expert Rev Clinical Dermatology 6th edn; 24: 665. McGraw-Hill. at: http://www.who.int/lep/resources/expert_pdf/en/index.html. WHO Geneva.

Treatment Approaches. Curr Opin Infect Dis; 16:421427. Mol Med; 2002:114.