Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. The prevalence and number of new cases have recently markedly decreased in Korea, and the possibility of leprosy can therefore be clinically overlooked. However, leprosy is still endemic in various regions of the world. A 30 year-old male immigrant from Indonesia presented with an erythematous plaque without sensory loss on his face six months after immigration. The skin lesion was diagnosed as tuberculoid leprosy based on clinico-pathology.
Emigrants and Immigrants* ; Emigration and Immigration ; Granulomatous Disease, Chronic ; Humans ; Indonesia ; Korea ; Leprosy ; Leprosy, Tuberculoid* ; Male* ; Mycobacterium leprae ; Prevalence ; Skin

Humans ; Leprosy, Tuberculoid ; diagnosis ; pathology ; Male ; Middle Aged ; Toes ; pathology

INTRODUCTION: Along with the remarkable decrease in incidence of Hansen's disease, many physicians have little experience and are unfamiliar with the disease entity. Consequently physicians may make an error in or delay the correct diagnosis more frequently in these days. We feel that the physicians should constantly be interested in Hansen's disease, and be aware of the updated aspects as compared with the past. MATERIAL AND METHODS: We have analysed 13 new patients of Hansen's disease in Busan, Ulsan, and Kyungnam district during last 6 years (2002-2007). The clinical features of 13 new patients were compared with 370 cases reported 30 years ago, in our clinic. RESULTS: Among 13 patients, male was 4 and female was 9. Mean age was 63 years, and the mean duration until diagnosis was 15.6 months. Two patients had familial history of Hansen's disease. In the classification, lepromatous leprosy was 6, borderline lepromatous leproys was 4, mid-borderline leprosy was 1, borderline tuberculoid leprosy was 1, and tuberculoid leprosy was 1. Eight patients of 13 have experienced lepra reaction. Level of BI and PGL-I antibody corresponded with type of the disease. Among 13 patients, 9 patients (69.2%) were resident in Busan. CONCLUSION: There was an increase in female population, and mutibacillary forms of Hansen's disease were more common as compared with the past. Since majority of patients were resident in Busan and the duration until diagnosis was too long, the dermatologist, especially in Busan should be interested in and carry on clinical studies of Hansen's disease constantly.
Female ; Humans ; Incidence ; Leprosy ; Leprosy, Lepromatous ; Leprosy, Multibacillary ; Leprosy, Paucibacillary ; Leprosy, Tuberculoid ; Male

BACKGROUND: Leprosy is an infectious disease with two polar forms, tuberculoid leprosy (TT) and lepromatous leprosy (LL), that are characterized by strong cell-mediated immunity (CMI) and CMI anergy, respectively. Transforming growth factor-beta (TGF-beta) is a family of growth factors involved in essential physiological processes, including development, differentiation, tissue repair, cell growth control and inflammation. Cellular signaling by TGF-beta family members is initiated by the assembly of specific cell surface receptors that activate transcription factors of the Smad family. Deregulation of the TGF-beta-Smad signaling pathway has been implicated in developmental disorders and several human diseases. Recently, ELISA & immunohistochemistry revealed high expression of TGF-beta isoforms in LL. OBJECTIVE: The purpose of this study was to investigate TGF-beta-Smad signaling in various forms of leprosy. METHODS: We investigated the involvement of TGF-beta by immunohistochemical staining for Smad 2 and 3 in skin biopsies from six patients of BL and four patients of TT. RESULTS: The inflammatory cells, keratinocytes and fibroblasts in BL showed strong positivity for both Smad 2 and 3, whereas those in TT showed little positivity. CONCLUSION: The high expression of Smad 2/3 in BL could represent high expression of TGF-beta, which possibly contributes to local CMI anergy and other clinical characteristic features of leprosy.
Biopsy ; Communicable Diseases ; Enzyme-Linked Immunosorbent Assay ; Fibroblasts ; Humans ; Immunity, Cellular ; Immunohistochemistry ; Inflammation ; Intercellular Signaling Peptides and Proteins ; Keratinocytes ; Leprosy* ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Physiological Processes ; Protein Isoforms ; Receptors, Cell Surface ; Skin ; Transcription Factors ; Transforming Growth Factor beta

BACKGROUND: On previous reports, the incidence of hepatitis C in Korean leprous patients is too high (55.6~69%). We investigated the percentage of the leprous patients with hepatitis C, genotypes, and risk factors in National Sorokdo Hospital. METHODS: Out of total 714 leprous patients in National Sorokdo Hospital, 147 selected risky patients (with abnormal AST/ALT, plateletleprosy, 29 (5.57% out of 503) were patients with lepromatous leprosy and 16 (11.94% out of 134) were those with tuberculoid leprosy. Genotypic result for 41 patients was 1b in 16 (39.02%), 2a in 20 (48.78%), other type in 3 (7.32%) and mixed in 2 (4.88%). Only 2 of the patients (4.40%) had experienced blood transfusion before 1990, 20 (44.40%) had taken an operation or tattooing, 3 (6.67%) were given intravenous drugs, 2 (4.44%) were family members as married couple, and as much as 20 (44.40%) had no previously known possible risk factors. CONCLUSIONS: Major genotype of hepatitis C in leprous patients was 2a and it is different from that of other Korean population without leprosy (p-value= 0.0027). The percentage of patients with hepatitis C was not related to the subclass of the leprosy. Further studies for the non-blood transmission of hepatitis C virus in them and its controls are needed.
Blood Transfusion ; Female ; Genotype* ; Hepacivirus ; Hepatitis C* ; Hepatitis* ; Humans ; Incidence ; Leprosy ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Male ; Risk Factors* ; Tattooing

BACKGROUND: Histologic evidence of destruction of a cutaneous nerve by granulomatous inflammation is a feature of tuberculoid leprosy. However, identifying remnants of the nerve in the granuloma may be difficult in some instances because of morphologic similarities with granulomatous dermatoses, such as sarcoidosis, lupus vulgaris, tertiary syphilis, etc. OBJECT: The purpose of this study was to investigate the expressions of S-100 and EMA and to estimate area, thickness and density of nerves for differentiation tuberculoid leprosy from sarcoidosis. METHODS: Paraffin embedded tissues from patients of 11 tuberculoid leprosy, and 4 sarcoidosis were investigated for the expression of S-100, EMA and for the measurement of area, thickness, density of nerves by image analysis. RESULTS: S-100 staining in tuberculoid leprosy demonstrated fragmented, infiltrated, swollen nerves or the absence of dermal nerves(45.5%). All of the detectable nerves were identified within granulomas from tuberculoid leprosy, but nerves from sarcoidosis were widely distributed over inside(33.3%) or outside of the granulomas, perivascular, and periappendageal areas. There was no expression of EMA in skin lesions from leprosy, sarcoidosis, even in the site showing perineural thickening in S-100 stain. The cross-section area and the thickness of nerves were decreased in order of tuberculoid leprosy, sarcoidosis, and normal control group(p<0.05). The density of nerves was decreased in order of normal control group tuberculoid leprosy, and sarcoidosis with statistical significance between tuberculoid leprosy and sarcoidosis. CONCLUSION: It is possible to differentiate tuberculoid leprosy from sarcoidosis by the observation of the morphologic changes with S-100 protein stain and the measurement of the cross-section area, thickness and density with image analysis.
Diagnosis, Differential* ; Granuloma ; Humans ; Image Processing, Computer-Assisted* ; Inflammation ; Leprosy ; Leprosy, Tuberculoid* ; Lupus Vulgaris ; Paraffin ; S100 Proteins ; Sarcoidosis* ; Skin ; Skin Diseases ; Syphilis

BACKGROUND: Histologic evidence of destruction of a cutaneous nerve by granulomatous inflammation is a feature of tuberculoid leprosy. However, identifying remnants of the nerve in the granuloma may be difficult in some instances because of morphologic similarities with granulomatous dermatoses, such as sarcoidosis, lupus vulgaris, tertiary syphilis, etc. OBJECT: The purpose of this study was to investigate the expressions of S-100 and EMA and to estimate area, thickness and density of nerves for differentiation tuberculoid leprosy from sarcoidosis. METHODS: Paraffin embedded tissues from patients of 11 tuberculoid leprosy, and 4 sarcoidosis were investigated for the expression of S-100, EMA and for the measurement of area, thickness, density of nerves by image analysis. RESULTS: S-100 staining in tuberculoid leprosy demonstrated fragmented, infiltrated, swollen nerves or the absence of dermal nerves(45.5%). All of the detectable nerves were identified within granulomas from tuberculoid leprosy, but nerves from sarcoidosis were widely distributed over inside(33.3%) or outside of the granulomas, perivascular, and periappendageal areas. There was no expression of EMA in skin lesions from leprosy, sarcoidosis, even in the site showing perineural thickening in S-100 stain. The cross-section area and the thickness of nerves were decreased in order of tuberculoid leprosy, sarcoidosis, and normal control group(p<0.05). The density of nerves was decreased in order of normal control group tuberculoid leprosy, and sarcoidosis with statistical significance between tuberculoid leprosy and sarcoidosis. CONCLUSION: It is possible to differentiate tuberculoid leprosy from sarcoidosis by the observation of the morphologic changes with S-100 protein stain and the measurement of the cross-section area, thickness and density with image analysis.
Diagnosis, Differential* ; Granuloma ; Humans ; Image Processing, Computer-Assisted* ; Inflammation ; Leprosy ; Leprosy, Tuberculoid* ; Lupus Vulgaris ; Paraffin ; S100 Proteins ; Sarcoidosis* ; Skin ; Skin Diseases ; Syphilis

BACKGROUND: The recent experimental observations suggested that location of M. leprae in the Schwann cells was mediated by epineurial and endoneurial endothelial cells, giving M. leprae access to the inner compartment of nerves and thus to Schwann cells. CD1 is a family of nonpolymorphic beta2-microglobulin-associated transmembrane glycoproteins that is structurally related to classical MHC Ag-presenting molecules, but is encoded by a separate genetic locus. Recent reports have described that human T cells specifically recognize foreign lipid and glycolipid antigens presented by CD1 proteins. Thus, it appeared likely that CD1 represents the key component of a MHC - independent pathway for antigen presentation to T cells. OBJECTIVE: we observed expression of antigen presenting molecules, such as MHC class I, II and CD1, in the vascular endothelial cells and inflammatory cells of leprosy skin lesion. METHODS: MHC class I, II and CD1 expression were studied using immunohistochemical stains. RESULTS: 1. In immunohistochemical stain of HLA-A, B, C, the level of expression in vascular endothelial cells of the borderline tuberculoid leprosy is higher than that of borderline lepromatous leprosy, but lower than that of normal skin tissue. 2. In HLA-A,B,C expression of the inflammatory cells, the level of borderline tuberculoid leprosy is higher than that of borderline lepromatous leprosy and of normal skin tissue(p>0.05). 3. Expression levels of HLA-DP, DQ, DR on endothelial cells decrease significantly in order of normal tissue, borderline tuberculoid leprosy, borderline lepromatous leprosy, lepromatous leprosy(p<0.05). 4. Expression levels of HLA-DP, DQ, DR on inflammatory cells decrease in order of lepromatous leprosy, borderline lepromatous leprosy, borderline tuberculoid leprosy, normal tissue, but statistical significance did not exist. 5. In immunohistochemical stains of CD1b, 3 sections of all 4 normal skin sections and 1 section of 3 borderline tuberculoid leprosy sections showed focal positivity on the dermal inflammtory cells, but borderline lepromatous leprosy sections did not show any positive inflammatory cells. 6. Epidermal Langerhans cells showed positivity on immunohistochemical stains of CD1a and CD1b. CONCLUSION: These results suggest that expression of MHC class I and II on the vascular endothelial cells and expression of CD1b on the inflammatory cells decrease in order of immunity of lepromatous skin lesion and that vascular enothelial cells play an important role in the pathogenesis of leprosy.
Antigen Presentation ; Coloring Agents ; Endothelial Cells* ; Genetic Loci ; Glycoproteins ; HLA-A Antigens ; HLA-DP Antigens ; Humans ; Langerhans Cells ; Leprosy* ; Leprosy, Borderline ; Leprosy, Lepromatous ; Leprosy, Multibacillary ; Leprosy, Paucibacillary ; Leprosy, Tuberculoid ; Schwann Cells ; Skin* ; T-Lymphocytes

Leprosy, one of the oldest diseases known to humans, still affects almost 10-15 million people throughout the world. Neuropathy affects 15-20% of infected individuals. Therefore leprous neuropathy is still one of the most common neuropathy in the world. It is due to infection with Mycobacterium leprae and occurs primarily in Asia and Africa. The cardinal symptom of leprosy is sensory loss caused by superficial neuropathy. Anesthetic depigmented skin lesions are an important finding and should be sought. Nerve involvement is a hallmark of leprosy. Nerve involvement is rare in other diseases, so the finding of skin lesions with enlarged nerves should raise the possibility of leprosy. Nerve involvement tends to occur with skin lesions, and the pattern of nerve involvement parrellels the skin disease. In the tuberculoid form, mononeuropathy multiplex is typical pattern, whereas asymmetrical or symmetrical polyneuropathy is most common in the lepromatous form. Motor involvement occurs in a predictable sequence as a result of nerve trunk damage to those nerves that course close to the skin surface and hence are locally cool. Nerve involved include the ulnar nerve at the elbow, the deep peroneal branch at the ankle, superficial branches of the facial nerve, and the median nerve at the wrist, and especially the greater auricular nerve. Nerve involvement without skin lesions, called pure neural leprosy, can occur. Other characteristic findings are thickened nerve, trophic ulcers, mutilated digits, and Charcot joint. In clinically affected nerves, the motor nerve conduction velocities are minimally slow. The terminal latency is often prolonged and the compound muscle action potentials are temporally dispersed and decreased in amplitude. Sensory and mixed compound nerve action potentials are often difficult to obtain or else a reduced amplitude. The facial nerve is commonly involved in leprosy. Improvement in motor nerve conduction was reported in leprosy patients under sulfone treatment. Nerve biopsy reveals subperineurial edema and various amounts of loss of myelinated and unmyelinated fibers. Teased fiber studies reveal paranodal demyelination affecting successive internodes. Therefore segmental demyelination is the main pathology in leprous neuropathy.
Action Potentials ; Africa ; Ankle ; Arthropathy, Neurogenic ; Asia ; Biopsy ; Demyelinating Diseases ; Edema ; Elbow ; Facial Nerve ; Humans ; Leprosy ; Leprosy, Tuberculoid ; Median Nerve ; Mononeuropathies ; Mycobacterium leprae ; Myelin Sheath ; Neural Conduction ; Pathology ; Polyneuropathies ; Skin ; Skin Diseases ; Ulcer ; Ulnar Nerve ; Wrist

Recently, many reports suggest that the activation of Toll-like receptor 2 (TLR2) by microbial lipoproteins may initiate innate defense mechanism against infectious pathogens. Especially, TLR2 is critical in the immune response to mycobacterial infections and the mutations in the TLR2 have been shown to confer the susceptibility to severe infection with mycobacteria. A previous study reported that there is a mutation of TLR2 (TLR2-R677W) in lepromatous leprosy. In this study, thus, we performed the functional study on TLR2 by measurement of IL-12 production in serum and monocytes from leprosy patients with TLR2 mutation (R677W) to verify the relation between TLR2 and the susceptibility to leprosy. Tuberculoid leprosy patients showed higher serum levels of IL-12p70 compared with those of lepromatous leprosy. In contrast with the lepromatous leprosy patient with TLR2 mutation, especially, the group with wild-type TLR2 showed 2-fold increase levels of IL-12p70. Functional studies demonstrated that monocytes from patient with the TLR2 mutation, in comparison to the wild-type TLR2, is significantly less responsive to Mycobacterium leprae lysate. Our results reveal that TLR2 has a important role in the IL-12 production from monocytes and the susceptibility of lepromatous leprosy.
Humans ; Interleukin-12* ; Leprosy* ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Lipoproteins ; Monocytes ; Mycobacterium leprae ; Toll-Like Receptor 2