Case report Date Moderator Consellor Oponen

: Borderline lepromatous (BL) type of leprosy presenting as single cutaneous macule : March 25th 2013 : dr. Satya Wydya Yenny, Sp.KK : dr. Sri Lestari, SpKK(K), FAADV, dr. Satya Wydya Yenny, Sp.KK : dr. Henry Tanojo, dr. Tutty Ariani

Frien Refla Syarif PPDS IK. Kulit dan Kelamin FK. Unand / RS. Dr. M. Djamil Padang

Borderline lepromatous (BL) type of leprosy presenting as a single cutaneous macule

ABSTRACT Background: Localized lepromatous or BT disease is a rare presentation of multibacillary leprosy. Borderline lepromatous leprosy usually presents with many widely distributed skin lesions. Most patients with BL leprosy have numerous small lesions with central infiltration. Tuberculoid leprosy presents on the skin as macules or plaques, which are usually solitary or few in number. Borderline tuberculoid leprosy manifests as skin lesions that resemble those of tuberculoid leprosy, but tend to be more numerous. Case: A 48 years old woman with numbness solitary lesion on the anterior left lower leg came to outpatient Dermato-Venereology Department of dr.M.Djamil Hospital. On clinical feature first suspected differential diagnosis were tinea corporis and suspected nevus spilus. We did the hydroxy-potassium examination but there were no fungi elements found and there were no signs and symptoms of nevus spilus resulted from Skin Tumor and Skin Surgery Division. Though our patient presented with clinical features suggestive of tuberculoid leprosy, histopathology of a skin biopsy from her solitary lepromatous lesion confirmed BL type of leprosy. We treat the patient with MDT PB. Keywords: borderline lepromatous type leprosy, single cutaneous macule ABSTRAK Latar belakang: Localized lepromatous atau lepra tipe BT jarang memiliki gejala klinis sebagai lepra multibasiler. Gejala klinis lepra tipe BL biasanya muncul dengan lesi yang terdistribusi luas dan banyak. Kebanyakan pasien lepra tipe BL memiliki banyak lesi-lesi kecil dengan infiltrate sentral. Lepra tipe tuberkuloid muncul sebagai makula atau plak pada kulit, di mana sering soliter atau berjumlah sedikit. Lepra tipe borderline tuberkuloid bermanifestasi sebagai lesi kulit yang bisa berkembang menjadu lepra tipe tuberkuloid dan cenderung bertambah banyak. Kasus: Seorang wanita berusia 48 tahun dengan lesi tunggal pada tungkai bawah bagian anterior yang terasa mati rasa datang ke Poliklinik Bagian Kulit dan Kelamin RSUP dr. M. Djamil. Riwayat klinis yang didapat, awalnya kami mendiagnosa banding dengan tinea korporis dan nevus pigmentosus. Kami lakukan pemeriksaan menggunakan KOH namun tidak ditemukan elemen jamur dan hasil konsultasi Divisi Tumor Kulit dan Bedah Kulit menyatakan tidak ada tanda dan gejala dari nevus pigmentosus. Walaupun pasien memiliki klinis lepra tipe tuberkuloid, hasil pemeriksaan histopatologinya adalah lepra tipe BL. Kami memberikan terapi MDT PB pada pasien. Kata kunci: lepra tipe borderline lepromatous, makula soliter

INTRODUCTION

Leprosy is a chronic infectious disease caused by Mycobacterium leprae. The bacteria is an intracellular, obligate, acid-fast bacillus affects the peripheral nerves, skin, mucosa of upper respiratory track, muscle, bone, and testes, but not central nerve system.1,2 Leprosy is a chronic disease with a long incubation period. An average incubation time of 2-5 years has been calculated for tuberculoid cases and 8-12 years for lepromatous cases.1,2 Leprosy is a primarily disease of developing countries. The geographic distribution of this disease is contributed by low socioeconomic condition, poor hygiene, and cold climate. It is more common in men than in women by a 2:1 ratio.3,4 Leprosy is diagnosed by definite loss of sensation (hypo esthetic or anesthetic) in solitary or multiple hypopigmented or reddish skin lesions, peripheral nerve changes such as nerve enlargement, sensory impairment, anhidrosis on palms or soles, or presence of acid-fast bacilli on slit skin smear.5 The immunological response to M. leprae mounted by the host will determine the different potential clinical states.1 Ridley-Jopling classified leprosy, based on clinical, bacteriology, histopathology, and immunology features, into tuberculoid (TT), borderline tuberculoid (BT), borderline (BB), borderline lepromatous (BL), and lepromatous (LL).3 The Ridley-Jopling classification is used to differentiate types of leprosy and helps in determining the prognosis. A general classification of disease is based on the number of skin lesions present and the number of bacilli found on tissue smears. Paucibacillary disease (indeterminate leprosy [IL] and TT) has fewer than 5 lesions and no bacilli on smear testing. Five or more lesions with or without bacilli (borderline leprosy and LL) is considered multibacillary disease.3,5,6

CASE REPORT A 48 years old woman came to the Out Patient Clinic Dermato-Venereology Department of Dr. M. Djamil Hospital on February 2nd 2013, with:

Chief complaint : There was numbness whitish patch which surrounded with brownish patch on the front left lower leg since 1 year ago.

Present illness history: Initially, there was numbness whitish patch which surrounded by brownish patch appeared on the front left lower leg since 1 year ago. She complained about numbness on the whitish patch. History of dry skin on her legs and the body (+) There were no new numbness whitish/reddish patches on her body, and there was no complaint about existing patches become red. She never complained for fever, malaise, and joints pain. There was no history of reduced hand and foot fingers and ulcer that difficult to be cured. There was no history of eyebrow baldness, eye dryness, eyelid closure disturbance, or eyesight disturbance. There was no history of traditional medication, no history of past leprosy medication. There was no history of clawed or mutilated fingers or complicated feet ulcers.

Past illness history: There was no history of white/ red patches on her body Complete immunization history (including BCG) when she was a kid There was no history of chronic cough History of close contact to person who had numbness whitish/reddish patches before was denied

Contact and family history: There was no history of contact with families, neighborhood, or close friends with anesthetic white or red patches on their skin, disabilities, long period history of MDT consumption, or complicated ulcers on their palms or soles. Social history: She was born and lives in Pariaman. She never moved out from the city. She is an elementary schools teacher. She lives with her family, living in a house of 6x10 meters, occupied by a total of 5 people. It has enough air circulation. Physical examination: Consciousness General state

: compos mentis cooperative : - Disease severity - Weight - Height - BMI : - heart rate - Blood pulse - Respiratory rate - Temperature : mild : 48 kg : 152 cm : 20,77 kg/m2 (normal weight) : 110/70 mmHg : 88 x/min : 18 x/min : afebris

Vital sign

General examination: Head Hair Eye Ear nose throat Mouth & teeth Neck Chest - Lungs - Heart Abdomen Back Extremity Lymph nodes : no abnormality found : no abnormality found : anemic sclera (-), icteric conjunctiva (-) : no abnormality found : no abnormality found : no abnormality found : no abnormality found : no abnormality found : no abnormality found : no abnormality found : edema (-) (arms & legs) : no enlargement on cervical, axillary, or inguinal lymph nodes

Dermatology state: Location : anterior left lower leg Distribution Shape Arrangement Border Size Efflorescence Palpation Sensibility tests: Pain test Touch test Temperature test : localized : not specified : non specified : well defined : plaque : whitish macule which surrounded by brownish macules : smooth surface

Lesion counts : 1

: hypo esthetic on lesions on her anterior left lower leg : hypo esthetic on lesions on her anterior left lower leg : hypo esthetic on lesions on her anterior left lower leg

Peripheral nerves examination: Greater auricular nerves: not palpable Radial nerves: not palpable Ulnar nerves: not palpable Median nerves: not palpable Lateral popliteal nerves: not palpable Posterior tibial nerves: not palpable Muscle strength testing (MRC scale): Orbicularis oculi muscles Abductor digiti minimi muscles Abductor policis brevis muscles Dorsal interossei muscles Posterior tibial muscles Miscellaneous examination: Contracture : (-) Mutilation : (-) Muscle atrophy: (-) Xerotic skin : (+) 5 :5 :5 :5 :5 :5

 Trophic ulcers : (-) Madarosis Claw hands Ape hands : (-) : (-) : (-) Lagophthalmus: (-)

Wrist drop : (-) Dropped foot : (-) Leonine face : (-) Summary: A 48-year-old woman, minang ethnic, a teacher presented with numbness whitish patch which surrounded by brownish patch appeared on the front left lower leg since 1 year ago. She complained about hypoesthetic whitish patch. History of dry skin on her legs and the body (+). New numbness whitish/reddish patches on her body, and there was no complaint about existing patches become red (-). History of fever, malaise, and joints pain (-). History reduce of hand and foot fingers and ulcer that difficult to be cured (-). History of eyebrow baldness, eye dryness, eyelid closure disturbance, or eyesight disturbance (-). History of past leprosy medication (-). History of clawed or mutilated fingers or complicated feet ulcers (-). She had complete immunization history (including BCG) when she was a kid. History of chronic cough (-). History of close contact to person who had numbness whitish/reddish patches before was denied. Physical examination was in normal limit. Dermatology state on her anterior left lower leg, there was a localized hypopigmented macule which surrounded by brownish macule with plaque in size. Sensibility tests were hypo esthetic on lesions on her anterior left lower leg. There was hypo esthetic sensibility on the lesion on her anterior left lower leg with no abnormality of muscle strength testing and thickened nerves were not found. Xerotic skin (+). Working diagnosis: Suspect TT leprosy Differential diagnosis: BT leprosy Tinea corporis Suspect nevus spilus Planning Hidroxy-potassium examination 6

 Consult to Skin Tumor and Skin Surgery Division Slit-skin smears (both earlobes and on the lesion) Histopathology examination (anterior left lower leg)

Result of hydroxy-potassium examination: There were no fungi elements found. Result of tumors and skin surgery divisions consult: There were no signs and symptoms of nevus spilus. Result of slit-skin smears Right earlobes Left earlobes

: there were no solid bacilli, no fragmented/granular bacilli found : there were no solid bacilli, no fragmented/granular bacilli found

Anterior left leg lesion : there were no solid bacilli, no fragmented/granular bacilli found Result of histopathology examination Epidermis tissues atrophy. In the upper dermis appear the granulomas containing epithelioid macrophages with cytoplasm, granuler and vacuolated and few lymphocytes. Below of dermis tissues fibrocollagen with little gland, the granuloma perivaskuler, and perineural periapendik. BTA was not found. Interpretation: Morbus Hansen BL type Routine blood count Hemoglobin White blood cells Hematocrit Platelets

: 12,1 g/dL : 6.800 /mm3 : 40% : 324.000 (N: 5000-10000) (N: 38-43%) (N: 150.000-400.000)

Diagnosis: Polar tuberculoid leprosy General treatment: Explain to the patient regarding her disease (course of leprosy, leprosy transmission, reactional state, prognosis, complication, side effects of drugs, and physicial disability possibility).

 Educate the patient the importance to take routine medicines and to check-up regularly until healed and explain to the patient that her disease needs long period of therapy. Explain to the patient the adverse reaction might be happen but do not to stop MDT therapy. Educate the patient the value of careful prevention of disability and types of disability that may occur in the future. Specific treatment Systemic therapy: MDT PB (rifampin 600 mg/month (supervised); dapsone 100 mg/day) Neurotropic vitamin 1x1 tab Topical therapy: Urea 10% twice a day after taking a bath to all over her body Prognosis: Quo ad vitam

: bonam

Quo ad sanationam : dubia ad bonam Quo ad cosmeticum : bonam Quo ad functionam : dubia ad bonam

DISCUSSION We reported a case of 48 years old woman with chief complaint numbness whitish patch which surrounded by brownish patch on her anterior left leg since 1 year ago. From anamnesis we got, she has the cardinal sign of leprosy which is the numbness lesion. From physical examination there was hypoesthetic sense on the lesions. The first time we saw the lesion, we suspected it was a tinea corporis with numbness sense being ignored, then we did the hydroxy-potassium examination from scratched lesion but the result was negative. We also suspected this lesion was a nevus spilus from the appearing of the lesion, but after we consulted to the divison of skin tumor and skin surgery, there were no signs and symptoms of the suspected disease. After that we did the slit-skin smears on right earlobes, left earlobes and anterior left leg lesion with result: there were no solid bacilli, no fragmented/granular bacilli found. From the anamnesis and physical examination we found numbness and hypoesthetic sense on the lesion 8

we did skin biopsy on the lesion for examining histopathology and the result was: epidermis tissues atrophy. In the upper dermis appear the granulomas containing epithelioid macrophages with cytoplasm, granuler and vacuolated and few lymphocytes and below of dermis tissues fibrocollagen with little glands, granuloma perivaskuler, and perineural periappendic. The interpretation of this examination was Morbus Hansen BL type. Skin lesions are few in TT type leprosy. One erythematous large plaque is usually present, with well-defined borders that are elevated and that slope down into an atrophic center. The lesions can become arciform or annular. They can be found on the face, limbs, or elsewhere, but they spare intertriginous areas and the scalp. Lesions can be dry and scaly, hypohidrotic, and hairless. Another presentation involves a large, asymmetric hypopigmented macule. Both types of lesions are anesthetic and involve alopecia. Spontaneous resolution can occur in a few years, leaving pigmentary disturbances or scars. Progression can also occur, leading to borderline-type leprosy. In rare instances in which a patient is untreated for many years, the lepromatous type can develop. Neural involvement is common in TT; it leads to tender, thickened nerves with subsequent loss of function. The great auricular nerve, common peroneal, ulnar, radial cutaneous and posterior tibial nerves are often prominent. Nerve damage can happen early, resulting in wrist drop or foot drop.3,5,6 Borderline tuberculoid leprosy (BT): Lesions in this form are similar to those in the tuberculoid form, but they are smaller and more numerous. The nerves are less enlarged and alopecia is less in BT than in other forms. Disease can remain in this stage, it can convert back to the tuberculoid form, or it can progress to LL.3,5,6 Borderline lepromatous leprosy (BL): Lesions are numerous and consist of macules, papules, plaques, and nodules. Annular punched-outappearing lesions that look like inverted saucers are common. Anesthesia is often absent. As with the other forms of borderline leprosy, the disease may remain in this stage, improve, or regress.3,5,6 Current WHO recommendations for treatment of leprosy are as follows: Paucibacillary disease: Dapsone 100 mg/d plus rifampin 600 mg once a month for 6 months. Multibacillary disease: Dapsone 100 mg/d plus rifampin 600 mg once a month plus clofazimine 300 mg once a month and 50 mg/d for 1 year. Single skin lesion: A single dose of rifampin 600 mg, ofloxacin 400 mg, and minocycline 100 mg.3,5,7,8 We gave this patient MDT PB based on the clinical sign with WHO recommendation. The histopathology examinations result was BL type of leprosy confusing the diagnosis. We didnt give ROM (rifampisin, ofloxacyn and minocycline) because of the result of histopathology examination revealed the BL type of leprosy. The host immune response to Mycobacterium leprae is critical for controlling the infection, but is also responsible for the immunopathological damage that may develop in nerves and specific organ sites.9 In LL disease, a lack of cell-mediated immunity against M. leprae 9

permits unrestricted bacillary proliferation, usually with the development of numerous cutaneous papules, nodules and plaques containing abundant bacilli and affecting much of the skin surface area including the face, ears, trunk and extremities.3,11 There may also be diffuse, fine or gross infiltration of the skin and widely disseminated, multiorgan disease may occur.3 The clinical presentation in our patient was consistent with tuberculoid leprosy, but histopathologically and her case was shown to be lepromatous. The presentation of LL or BL as a single cutaneous lesion (or localized lesions) is a rare variant of multibacillary disease4 and to our knowledge there are only seven similar cases reported in the literature.11-14 The expression of multibacillary leprosy as localized lepromatous disease is a recognized but unusual presentation of leprosy. The lesions should have a very high bacterial index, while the rest of the skin is negative, or nearly so, to standard methods of examination for M. leprae.15 Possible explanations postulated for this presentation include direct exposure of skin to M. leprae, coupled with repeated trauma, low skin temperature and local immunological causes.12,14

CONCLUSION We reported a case with a presentation of BL as a solitary lesion in 48 years old woman. This case remains rare and reinforces that certain aspects of the host cell-mediated response and pathophysiology of this important disease are still not fully understood. It is important that patients are classified correctly in the leprosy spectrum, so that they may receive the most appropriate treatment. The literatures of cases of BL type of leprosy presenting one single lesion are also few.

REFERENCES 1. Meyers WM. Leprosy. In: Guerrant RL, Walker DH, editors. Tropical infectious disease: principles, pathogenesis and practice. 2nd end. Philadelphia: Elsevier; 2006. p. 437-45. 2. Burdick AE, Copo VA, Frankel S. Leprosy. In: Tyring SK, Lupi O, Henne UR, editors. Tropical dermatology. Philadelphia: Elsevier; 2006. p. 256-66. 3. Rea TH, Modin RL. Leprosy. In: Freedberg IM, Eisen AZ, Wolff AK, Austen KF, Goldsmith LA, Katz SI, Fitzpatrick TB, editors. Dermatology in general medicine. 7th ed. New York: Mc.Graw-Hill; 2008. p. 1786-96. 4. Halim PW, Rudiyanto. Diagnosis dan pengobatan penyakit lepra. In: Soewono JPH, Suparniati E, editors. Pedoman standar pengobatan dan pengelolaan lepra di rumah sakit lepra Sitanala. Tangerang. 2000. p. 13-38. 10

5. Silva MR, Castro MC. Leprosy .In : Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. First edition. Edinburgh, Mosby, 2003: 1145-52. 6. Gupta SK, Nigam S, Mandal AK, Kumar V. S-100 as a useful auxiliary diagnostic aid in tuberculoid leprosy. J Cutan Pathol. Jul 2006;33(7):482-6. 7. World Health Organization. Global leprosy situation, 2006. Wkly Epidemiol Rec. Aug 11 2006; 81(32):309-16. 8. World Health Organization. WHO-recommended MDT regimens. World Health Organization. Available at http://www.who.int/lep/mdt/regimens/en/. 9. Britton WJ. Immunology of leprosy. Trans. R. Soc. Trop Med. Hyg. 1993; 87: 50814. 10. Jha PK, Talwar S, Suresh MS, Panvelkar V. Localised borderline lepromatous leprosy. Lepr. Rev. 1991; 62: 21216. 11. Job CK, Kahkonen ME, Jacobsen R, Hastings RC. Single lesion subpolar lepromatous leprosy and its possible mode of origin. Int. J. Lepr. Other Mycobact. Dis. 1989; 57: 1218. 12. Yoder LJ, Jacobson RR, Job CK. A single skin lesion an unusual presentation of lepromatous leprosy. Int. J. Lepr. Other Mycobact. Dis. 1985; 53: 5548. 13. Mohammed KB. Lepromatous leprosy presenting as a single doughnut-shaped lesion on the face. Br. J. Dermatol. 1998; 138: 5601. 14. Misra RS, Ravi S, Iyengar B, Nath I. A histopathological and immunological profile of a single lesion lepromatous leprosy (LLs). Int. J. Lepr. Other Mycobact. Dis. 1991; 59: 6458. 15. Pfaltzgraff RE, Ramu G. Clinical leprosy. In: Hastings RC (ed.). Leprosy, 2nd edn. Edinburgh: Churchill Livingston, 1994; 23787.

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