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Laporan Kasus

ERYTHEMA NODOSUM LEPROSUM: CLINICAL FEATURES AND
HISTOLOGICALLY, REPORTED THREE CASES
St. Musafirah, Sri Vitayani M, Muh. Dali Amiruddin1, Mahmud Ghaznawie2
*

Departement of Dermato-Venereology, Medical Faculty of Hasanuddin University/
dr.Wahidin Sudirohusodo General Hospital, Makassar, Indonesia
** Departement of Pathology Anatomy, Medical Faculty of Hasanuddin University, Makassar, Indonesia

RINGKASAN
Reaksi Erythema Nodosum Leprosum (ENL) dapat terjadi pada pasien kusta, sebelum, selama dan sesudah
terapi. Reaksi tersebut dapat menyebabkan sekuele yang disebut sebagai deformitas. Dilaporkan 3 kasus kusta
disertai reaksi ENL, dengan gambaran klinis berbeda dan dikonfirmasi dengan pem eriksaan histopatologik.
Kasus 1, ENL muncul pada penderita kusta setelah pengobatan Multi Drug Therapy (MDT) dan Kasus 2 muncul
pada penderita selama pengobatan MDT. Kedua penderita tersebut, memberikan gambarn klinis identik dengan
adanya nodul erytema dan demam, secara histopatologik ditemukan gambaran karakteristik reaksi ENL Kasus 3,
reaksi ENL muncul pada penderita kusta sebelum pengobatan, dengan gambaran klinis adanya erosi dan ulcus
generalisata dan secara histopatologi ditemukan sebagai kusta lepromatous subpolar, dan ini merupakan kasus
yang tidak umum. Pada kasus 1 dan 2 diberikan terapi kortikostiroid oral dan memberikan perbaikan, akan tetapi
kasus ke 3 diberikan terapi kortikosteroid oral tetapi pasien meninggal dengan penyebab yang tidak diketahui.
(J Med Nus.2006;27:45-50)

SUMMARY
Erythema Nodosum Leprosum (ENL) reaction can appear in the leprosy patients before, during and after treatment.
The reaction may lead to neurologic sequele, then it will be deformities. We reported three cases of leprosy with ENL
reaction, whereas they have different clinical feature and have confirmed with histopathologically examination. Case
1, ENL appears to the leprosy patient after the treatment of Multi Drug Therapy (MDT) and the case 2, it appears to the
patients during tretament MDT. Both the patients, shown identical clinical featurs with pain nodul erythematous and
fever, and histopathologically was found characteristic as ENL reaction. The case 3, ENL reaction appears to the
leprosy patients before the treatment, clinical feature shown erosion and ulcer generalized and histopathologically
was found as leprosy subpolar. It is the uncommon case. Case 1 and 2 were given corticosteroid orally and some
improvement, eventhough case 3 also was given corticosteroid orally bu she died with unknown causes
* This paper had been read as oral presentation in Australasian Dermatopathology Society 26 th Annual Conference,
Brisbane Convention Centre, 26 – 28 August 2005 (J Med Nus.2006;27:45-50)

INTRODUCTION
All patients with leprosy are liable to develop acut,
subacute or protrated inflammatory episodes called
“lepra reaction”, which are the result of a sudden
alteration of the host-parasite relationship.1-4
Lepra reaction may be of two types (Ridley & Jopling,
1981): 1, 3-6
1.

Type 1 reaction is cell-mediated and occurs in
tuberculoid, boderline, and lepromatous leprosy.
There is inflammation of the existing leprosy
lesion. Histological examination will detect
improvement (reversal reaction) or worsening
(down grading reaction)

J Med Nus Vol. 27 No.1 Januari-Maret 2006

2.

Type 2 reaction occurs in lepromatous leprosy
and some BL cases. It is possibly an Arthus –
like phenomenon due to circulating immune
complexes.

3.

Type 3 reaction, is rare type, the Lucio
Phenomenon.

Erythema Nodosum Leprosum (ENL) , is named for
its most prominent clinical finding: an eruption of tender,
red nodules, 1,2,4 usually involve the face, trunk, and
extremities.7 It is an immune complex disease, a Type III
hypersensitivity reaction, and occurs almost exclusively
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and other organs these precipitates attract neutrophils.4. She push hospitalization with the reason she get lactation with 3 months old baby. Conclusion: ENL. and no anathesia. Adnexa. The reaction may lead to neurologic sequele. blood vessel walls. nerves.8 We reported three cases of leprosy with ENL reaction.7 Erythema Nodosum Leprosum (ENL) reaction can appear in the leprosy patients before. The follow up was done every 2 weeks and the corticostreoid oral tappered of 5 – 10 mg every 2 weeks.5.in patients with lepromatous leprosy (LLp and LLs) and only occasionally in patients with boderline leprosy (BL). with unknown causes. CASE REPORT Case 1 A 17 ys old woman. On histologically examination. Leprae in the globi form. J Med Nus Vol. She has been experinces approximately 1 year and more seriously in 1 month before came to the clinic. well-demarcated. ENL is characterized by vasculitis and panniculitis. neutrofil extent to subcutis layer. 27 No. Granuloma in dermis contain histiocyte cells and foamy cytoplasm.7. which precipitated in the tissue of the skin.1 Januari-Maret 2006 . In the upper extremities. There was enlarged on the facialis. and microabcess may form. 2. whereas they have different clinical feature and have confirmed with histopathologically examination. which further damage the tissue. we found that atrophy epidermis with thinned in subepidermal area. The treatment of the patient was given.2. that was found M. Polymorphonuclear leukocytes are predominant early. she passed out 3 weeks later. then it will be deformities. oral corticosteroid (metilprednisolon initially 40 mg/day to 2 weeks) and neuroroburamntia. during and after treatment.4. nerves and blood vessel were infiltrated of granuloma. In the physical examination. we found the ulcers and erosion simmetrically. Fite Faraco stain. ulnaris and peroneus nerves. The patien has not get MDT or other drugs.Unfortunately. came to the clinic dermatovenereology wahidin sudirohusodo general hospital with complain ulcers in the extremities and plaque 46 erythematous generalized.8 Histologically.1. Leprae and antibodies form immune complexes with complement. Skin smear for this case is (6+). there was plaque erythematous generalized with shine surface.7 Antigens of M.4. LLs type leprosy.

Fite Faraco staining: M. leprae absent. In the physical examination. LLs type . Skin smear of this case is negative. On histologically examination. The patients was improve after 6 weeks but the later weeks. Conclusion: ENL. LLs type leprosy. The follow up was done every 2 weeks and the corticostreoid oral tappered of 5 – 10 mg every 2 weeks. 10 days ago. In the physical examination. peeroneus and tibialis posterior nerves.Case 2 A 35 ys old man. neutrofil cells extent to subcutis layer. Leprae (+) fragmenetd. that was found M. The extremities was found anasthesia in. Fite Faraco stain. There was thickened and neuritis on the ulnaris and peroneus nerves. neutrofil until subcutis layer. The treatment of the patient similary of the patient’s before. Boderline Lepromatous. shiny and well-demarcated.The patients was improve after 10 months corticosteroid Case 3 A 57 ys old woman. J Med Nus Vol. Conclusion: ENL. we found that atrophy epidermis. that was found M. came to the clinic dermatovenereology wahidin sudirohusodo general hospital with complain nodul erythematous on face and all of the body. and the fever and arthralgia. was given oral corticosteroid (metilprednisolon initially 40 mg/day to 2 weeks) and neuroroburantia. there was hyperpigmentation macule on the face. The patien have been finished MDT 6 months ago. The treatment was given oral corticosteroid (metilprednisolon initially 40 mg/day to 2 weeks) and neuroroburantia. On histologically examination. All of the number given corticosteroid in this patients was 18 weeks. Fite Faraco stain. 27 No. All of the number given corticosteroid in this patients was 18 weeks. nodul of the bodies appear again. and the fever and neuritis. limbs and extremities with simetris. Also we found hyperpigmentation macule anasthesi in the chest. back and extremities.. with granuloma in dermis which contain histiocyte cells and foamy cytoplasm. Conclusion: ENL. The patien has getting MDT 5 months. so that corticosteroid dose was increased. there was nodul erythematous generalisata. The follow up was done every 2 weeks and the corticostreoid oral tappered of 5 – 10 mg every 2 weeks. Also nodul erythematous generalisata. 2 weeks ago. Skin smear of this case is negative. came to the clinic dermatovenereology wahidin sudirohusodo general hospital with complain nodul erythematous on face and all of the body. with granulomas in nerves and adnexa which contain histiocyte cells and foamy cytoplasm. leprae (+) and fragmented form. There was enlarged and neuritis on the ulnaris.1 Januari-Maret 2006 47 . we found that atrophy epidermis.

Leprae fagmented J Med Nus Vol.HISTOPHATOLOGIC FEATURES granuloma M. Leprae solid 48 M. 27 No.1 Januari-Maret 2006 .

according to severity.4. The granuloma without sitoplasma foamy (active lesion). the shown as LL. 2. Predominantly of leukocyte polymorphonuclear and may be microabhcesses form. End diagnosed as a lucio phenomenon.9 The prevalence of ENL was reported more than 50% in the lepromatouos leprosy and 25% in the borderline leprosy. and activation of peptic ulcers. which to give the corticosteroid such asa prednison or prednisolon is started with daily dose 40 – 80 mg. because there was bulla and ulcer in the upper extremitis that similar with pemphigus. such as coticosteroid. lucio leprosy appear often before treatment has been started.5. non-steroidal anti inflamati on (NSAID). 15. thalidomide. Langhans. Fite-faraco staining.14. Fite Faraco staining was found M. histologically was not appropriate with literatur.17 The nerve sheaths are laminated (onion peel appearance). and clofazimine. 27 No. 9-12 The reoccurance symptoms of ENL and consequence of dissability made it as health problem seriously. 5. and the second. AFB(+) in the cluster form. splinting and exercise and antibacterial therapy. all of the patients to improve with corticosteroid therapy. antimonial.19 Many of drug may be used to reduced to inflammation or as a anti inflammatory. 4.15 In the second and third cases. patient was die.2. A practical approach to management involves.15 Also.(18) Vasculitis and panniculitis are characterize.20 49 . and neutrophil cells extent to subcutis layers. infiltrate limphocyte polymorhonuclear and baccilli fragmented form. the most rapid control is essential. Usually 15-50% lepromatos leprosy is become ENL within the first year of treatment and 90% occur within the second year of treatment. with unknown causes. initial diagnosed as a ENL reaction. muscle wasting. it turned out of atrophic epidermis with thin of subepidermal area. development of Cushing’s syndrome with osteoporosis. They ware according to the literatur. 15 So that.5. paralysis and contracture.15 Also. histopattologic feature of ENL is leukocytoclastic vasculitis in artery or vein. Althought.4. Older lesions show vacuolated cytoplasm within macrophag due to lipid accumulation (lepra cells of Virchow).4.4. They agree with literature. The doses should be reduced to 40 mg after a few days or 5 – 10 mg/ week if the improve of neuritis.19-21 In this cases. Side effect of corticosteroid is suppress immune responses. The patients was not get drug yet. There was very solid granuloma that coat of all epidermis and dermis layer. 15.15-17 A macrophag granuloma is seen with no epitheloid cells and scanty lymphocytes and plasma cells unless there is complicating ENL.17 Managemnet of leprosy reaction should be: firstly. histologically as a lepromatous leprosy. we found thickened and neuritis on the ulnaris.1. Conclusion: lepromatous leprosy (LLp) with ENL. In the literature. Adnexa. 4.15 This is shown in clinical features of cases above. there is Infiltrate celluler axtensive that is almost invariably separated from the flattened epidermis by grenz zone. Early diagnosis and treatment and energetic management of reactional states should prevent to development of all disabilities. whereas it was mentioned that histologic features in the lucio phenomenon was found ischemic epidermal necrosis with necrosis of superficial blood vessels and oedema and endothelial proliferation of deeper vessels.1 Januari-Maret 2006 numerous and are found in packets (globi) within macrophages. Among granulomas there was much multinucleated Giant cells. that clinically ENL was commonly as pain nodules eryuthematous with fever and neuritis. pentoxifilline. leprae (+) and fragmented form.12.13 In the first cases. whether they have been get or not the treatment of MDT. histologic feature of lepromatous leprosy . sweat gland and nerve have been infiltrated by granulomas. peroneus nerves.15 Histologically this cases. But we found enlarged of ulnar and peroneus nerves. there was small pink lesion and plaque erythema in her trunk without anathesia.6. chloroquin.14 that is characterized by cutaneous hemorrhagic infarct in patients with diffuse infiltration of the skin by a granulomatous process heavily loaded with mycobacterium leprae.DISCUSSION Erythema Nodosum Leprosum is a disease that only appear in multibacciler leprosy patients. analgetic therapy. And then after the confirmed of histologically. we found epidermis atrophy and granulomas in dermis consisted of histiocyte cells with foamy cytoplasma. with clinical fatures was pain nodules erythematous generalised as well as fever and arthralgia. subtype of in a lepromatous leprosy (la lepra bonita) 3. in this case.19 In cases of neuritis. Also. to kill the bacilli and prevent extentsion of diseases. although corticosteroid was given in a long time in case 2 and three. 6. to control acut neuritis in order to prevent anasthesiaq. initial diagnosis as a pemfigus vulgaris.3. 17 Acid fast bacilli are J Med Nus Vol. According to the literature that a lucio phenomenon is a unusual type II reaction that is sometimes designated a type II reaction. And in patients 3. 15. anti-inflammatory therapy. long time follow up should be done to prevent occuring the side effect. After histolpatologic axamination. Other side effects include salt and water retention. No fever and arthralgia. Also.

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