Caracterización de lesiones bucales de pacientes con enfermedad de Hansen

Background and objectiveHansen disease, or leprosy, is caused by Mycobacterium leprae or Mycobacterium lepromatosis. Because these bacteria enter the body via the upper airways, they generate clinical manifestations in the nasal mucous membranes and the mouth. We aimed to describe the characteristics of oral lesions in patients with Hansen disease.Material and methodsCross-sectional observational study of 100 patients diagnosed with Hansen disease. We examined the oral cavity and recorded clinical findings on a disease reporting form for each patient. We also included the histopathologic findings for lesions that required a biopsy. Samples suggestive of Hansen disease were processed with hematoxylin-eosin, Ziehl–Neelsen, and Fite–Faraco staining. Variables were analyzed, as appropriate, with the χ² test, the Mann-Whitney U test, or the Spearman correlation coefficient.ResultsSixty-seven men and 33 women were included. The mean (SD) age was 48.1±16.4 years. Thirty-four patients had oral lesions. Lesions related to Hansen disease were found in 9 patients. The locations were the hard palate and upper lip. Oral lesions were significantly more frequent in patients with lepromatous leprosy, borderline lepromatous leprosy (P = .029), and erythema nodosum leprosum (P = .031).ConclusionsThe frequency of oral lesions is low in Hansen disease. Such lesions present as lepromas and leprous plaques on the hard palate and upper lip.     

White lesions of the oral cavity

White lesions are frequently found during the examination of the oral cavity. Although some benign physiologic entities may present as white lesions, systemic conditions, infections, and malignancies may also present as white oral lesions. An appreciation of the many clinical entities that white lesions may represent is necessary if a differential diagnosis of white lesions is to be elucidated. The appreciation of subtle clinical findings associated with white lesions of the oral cavity permits clinicians to better care for their patients.     

误诊为系统性红斑狼疮的瘤型麻风一例

报告1例女性瘤型麻风.患者女,39岁,因多关节痛,面部及上肢酱红色丘疹,斑块半年就诊,伴有光敏,发热及乏力.实验室检查:尿蛋白强阳性、血红蛋白 97g/L,血小板51 × 109/L,补体C3为560 mg/L(850～1930 mg/L)、C4为103 mg/L( 120～360 mg/L)、ANA 1:160阳性.符合系统性红斑狼疮诊断条件,给予泼尼松50 mg/d、沙利度按100 mg/d及羟氯喹200 mg/d,治疗后系统症状改善,但皮损进行性加重,两年后逐渐出现上腭及鼻中隔穿孔等病变.最终经组织病理检查及抗酸染色确诊为瘤型麻风.提示瘤型麻风可以出现多系统损害,自身抗体阳性,必须警惕误诊为风湿性疾病.     

Differential diagnosis of leprosy in developing countries--the skin and oral cavity

There are some skin diseases which are frequently mistaken for leprosy and in consequence, often treated like it for a long period of time. The rate of false diagnoses is especially high in leprosy control programs of developing countries. However, a wrong diagnosis of leprosy can mean psychological disaster to the patient because of the stigma still attached to leprosy. According to the literature and our own observations in Sierra Leone, Ethiopia, and the Sudan, we present data on the differential diagnosis of leprosy concerning the skin and oral cavity.     

Eye lesions in leprosy

Out of 742 out-patients screened for ocular disease, 177 (24%) had eye lesions due to leprosy. These were more in the lepromatous spectrum of the disease and showed increasing trend with age of patient and duration of the disease. Madarosis was the commonest lesion (76%). The serious and sight threatening lesions like lagophthalmos, corneal anaesthesia, corneal opacities and ulcers, iritis and complicated cataracts constituted 8.22% of the lesions. Blindness due to corneal opacity and complicated cataract developed in 6 patients, constituting 3.4% of eye lesions with a prevalence rate of 0.8% among all the leprosy patients. Although the blinding lesions occurred in a very small percentage of patients, most of these are preventable through early recognition and institution of appropriate treatment. The simple techniques of examination to detect potentially sight threatening lesions should be taught to all leprosy workers to prevent blindness among leprosy patients.     

A clinical study of eye complications in leprosy

A study was conducted to find out the incidence of ocular complications in leprosy. The ocular lesions were found in 6.6% of lepromatous leprosy and 1.6% in nonlepromatous leprosy. Out of 150 patients with eye lesions 74% were males and 80% belonged to lepromatous leprosy. The age group in all the patients varied from 3rd to 6th decade. Mean duration of leprosy ln lepromatous leprosy was 6.2 years. The important eye complication observed were lagophthalmos (8.1%), corneal ulcers (10%) and iridocyclitis (24%). The loss of eye-brows (76%) was found to be most frequent in this study followed by corneal lesion (62%). It is concluded that examination of eyes are essential in all types of leprosy.     

Molluscum contagiosum-like lesions in lepromatous leprosy

A 32-year-old male patient presented with multiple, asymptomatic, papulonodular lesions some of which were umbilicated simulating the clinical features of molluscum contagiosum. On examination the patient was found to be suffering from lepromatous leprosy. Slit-skin smear showed a BI of 6+ and histopathological examination of the nodular lesion showed features of lepromatous leprosy. The patient was treated with MDT-MB and improved remarkably in a few months.     

Generalized anetoderma in a patient with HIV and dual mycobacterial infection

A middle-aged HIV infected man receiving treatment for pulmonary tuberculosis, presented with a febrile illness along with evanescent, erythematous nodular lesions all over the body. On examination, he had features suggestive of lepromatous leprosy with lesions of erythema nodosum leprosum. In addition, there were multiple small, circumscribed areas of slack skin, clinically and histopathologically suggestive of anetoderma. Both leprosy and HIV infection are known to give rise to lesions of anetoderma. Pathogenesis of anetoderma in these infectious conditions is discussed.     

Inter-observer reliability in assessment of sensation of skin lesion and enlargement of peripheral nerves in leprosy patients

The accurate diagnosis of leprosy is important to both individuals and to the community. The diagnosis of leprosy is based on clinical examination. However, the reliability of clinical assessment of sensation in skin lesions and thickness of peripheral nerves on palpation has not been well studied, due to the lack of a gold standard. We report an inter-tester reliability study of the clinical assessment of skin lesions and thickness of ulnar and popliteal nerves in leprosy patients by different staff. For sensory testing of skin lesions, the agreement between the leprologist and leprosy control staff, and between one student and leprosy control staff, was poor (kappa values < 0-4). The agreement between the leprologist and the two students, between the two students, and between the other student and local leprosy control staff were fair (kappa values > 0.4, but < 0-6). For the palpation of ulnar and popliteal nerves, the agreement ranged from 0.36 to 0.52 and from 0.02 to 0.29 in different pairs of testers, respectively. The reliability of clinical diagnostic skills based on both sensory testing of skin lesions with the cotton wool method and palpation of superficial peripheral nerves was unsatisfactory.     

Verruciform Xanthoma: Report of Five Cases

We describe five cases of verruciform xanthoma (VX). The patients, all males, presented with single warty verrucous lesions of 0.5–2 cm size that had been diagnosed clinically as viral warts (four cases) and leukoplakia (one case). Two patients had the lesion in the oral cavity, two on the genital mucosa, and one on the scrotal skin. Histopathology was diagnostic, with verrucous and papillomatous epidermal hyperplasia with the silhouette of a viral wart but with numerous foamy histiocytes packed in the elongated dermal papillae. Columns of deep parakeratosis and neutrophils in the upper spinous layers, with a dermal plasma cell infiltrate were the other histopathologic findings. Excision of the lesions was curative, without recurrences, in the two patients who had lesions in the oral cavity; follow-up was not available in the cases with genital lesions. VX is an uncommon but distinctive clinicopathologic entity affecting the oral and genital mucosa that may be mistaken for benign, premalignant, and malignant conditions. VX can be diagnosed with certainty only on histopathologic examination.     

Clinical management of common oral lesions

Soft tissue lesions are among the most commonly occurring pathologic conditions in the oral cavity. The causes of these lesions can vary from immunologic and viral reactions to underlying systemic disease, dermatologic lesions, or neoplasms. The diagnosis of these conditions is usually based on the history, clinical appearance, and results of diagnostic procedures such as smears, culture, or examination of biopsy specimens when indicated. This paper will discuss the diagnosis and management of some of the most commonly occurring oral lesions, such as recurrent aphthous ulcers, herpetic ulcers, and candidiasis.     

Diagnostic exploration of enlarged peripheral nerves in suspected cases of leprosy. An analysis of 55 cases.

In 55 cases presenting with enlarged peripheral nerves without any skin lesions, a rice grain-sized biopsy of the nerve lesion was taken for histopathological examination. As a result definitive diagnoses could be established: leprosy was diagnosed in 32 cases. In 23 cases the cause of nerve enlargement was not leprosy: post-traumatic neuritis 9, cysts 5, hypertrophic neuritis 3, nonspecific 4, neurofibroma 1, and amyloidosis 1. In all of these cases there was a deficit of the nerve function and postoperatively there were no complications. The authors, as a result of this experience, believe that surgical exploration and biopsy is a harmless diagnostic tool for establishing a definitive diagnosis of leprosy in cases presenting with enlarged peripheral nerves without any skin lesions. In 23 out of 55 such cases the nerve enlargement was proved to be other causes than leprosy.     

Oral melanoma and other pigmentations: when to biopsy?

Oral pigmentations (OPs) are often neglected, although a meticulous examination of the oral cavity is important not only in the diagnosis of oral melanoma, but also for the detection of important clinical findings that may indicate the presence of a systemic disease. OPs may be classified into two major groups on the basis of their clinical appearance: focal and diffuse pigmentations, even though this distinction may not appear so limpid in some cases. The former include amalgam tattoo, melanocytic nevi, melanoacanthoma and melanosis, while the latter include physiological/racial pigmentations, smoker's melanosis, drug‐induced hyperpigmentations, postinflammatory hyperpigmentations and OPs associated with systemic diseases. We will discuss the most frequent OPs and the differential diagnosis with oral mucosal melanoma (OMM), underlining the most frequent lesions that need to undergo a bioptic examination and lesions that could be proposed for a sequential follow‐up.     

Lepromatous leprosy,melanoma, and basal cell carcinoma: clinical-histopathologic association

Cutaneous neoplasms frequently occur in leprosy, but there are few reports of the coexistence of leprosy and basal cell carcinoma in the same lesion. This case reports a 49-year-old male with an ulcerated plaque on the right lateral nasal wall, bright papules on the sternal region, and a blackened plaque on the right temporal region. The nasal and temporal lesions were diagnosed by histopathology as basal cell carcinoma and melanoma, respectively. The sternal lesions were excised with the repair of the “dog ear” which histopathological examination showed macrophages in the dermis parasitized with acid-fast bacilli, confirming the diagnosis of lepromatous leprosy with Fite-Faraco staining. This case report highlights the importance of referring the dog-ear specimen for histopathologic analysis.     

Diffuse leprosy of Lucio and Latapí: a histologic study

BACKGROUND AND PURPOSE: Ladislao de la Pascua described the spotted or lazarine leprosy for first time in 1844. Later on, Lucio and Alvarado studied and published it with the same names in 1852. Latapí re-discovered it in 1938 and reported it as 'Spotted' leprosy of Lucio in 1948. Frenken named it diffuse leprosy of Lucio and Latapí in 1963. Latapí and Chévez-Zamora explained that the fundamental condition of this variety of leprosy was a diffuse generalised cutaneous infiltration, naming it pure and primitive diffuse lepromatosis, upon which necrotising lesions develop, calling these lesions Fenómeno de Lucio or erythema necrotisans. A great number of histopathological reports have addressed the study of Lucio's phenomenon, and few about the histologic changes that take place in the course of diffuse lepromatous leprosy. The purpose of this work is to report the histologic findings observed in the study of 170 cutaneous biopsies of diffuse leprosy of Lucio and Latapí and 30 of Lucio's phenomenon. METHODS: This is a retrospective study, which included the examination of 200 biopsy skin specimens from 199 patients with diffuse leprosy at different course of the disease. These cases were diagnosed in Mexico from 1970 to 2004. RESULTS: The histologic examination revealed a vascular pattern affecting all cutaneous vessels, characterised by five outstanding features: a) colonisation of endothelial cells by acid-fast bacilli, b) endothelial proliferation and marked thickening of vessel walls to the point of obliteration, c) angiogenesis, d) vascular ectasia, and e) thrombosis. Necrotising lesions seen in diffuse lepromatous leprosy displayed two histopathological patterns: one of them, non-inflammatory occlusive vasculopathy and, the other one, occlusive vasculopathy, leukocytoclastic vasculitis, large neutrophilic infiltrate and lobular panniculitis. The first appeared as a result of the course of the occlusive vasculopathy produced by the colonisation of endothelial cells by Mycobacterium leprae. The second, as a result of a previous occlusive vasculopathy plus a leprosy reaction which is considered here as variant of ENL. CONCLUSIONS: Endothelial cell injury appears to be the main event in the pathogenesis of diffuse leprosy of Lucio and Latapí. Once M. leprae has entered the endothelial cell, the micro-organism damages the blood vessels, leading to the specific changes seen in this variety of lepromatous leprosy.     

Comparison of two systems of classification of leprosy based on number of skin lesions and number of body areas involved--a clinicopathological concordance study

BACKGROUND AND AIMS: WHO guidelines classify leprosy patients for therapeutic purposes into paucibacillary (PB) and multibacillary (MB) leprosy based on the number of skin lesions. An alternative system of classification has been in practice in Nepal from 1985 onwards, based on the number of body areas involved in patients of leprosy. We attempted a clinicopathological approach for comparison of these two systems of classification in leprosy patients for their ability to demarcate patients into groups of PB and MB leprosy. MATERIALS AND METHODS: The study included 108 leprosy patients (80 males and 28 females). Complete clinical examination and body charting was carried out in each patient noting the count of skin lesions and the number of body areas involved. Slit skin smears and skin biopsies were taken from an active skin lesion in all patients. RESULTS: On analysis, it was observed that there was good clinicopathological correlation between patients with 5 or < 5 skin lesions and 2 or < 2 body areas involved. (Clinical 95% and histological 96%) A similar correlation was also observed in the other group of patients with > 5 skin lesions and > 2 body areas involved, (Clinical 94% and histological 96%). There were almost identical numbers of patients represented in these two groups of classification. CONCLUSIONS: Our findings suggest that patients with involvement of 2 or less body areas can be classified as PB leprosy and those with more than 2 body areas involved can be classified as MB leprosy for the purposes of therapy. The study of areas of involvement in leprosy patients not only provides additional patient information but also adds another parameter as a basis for the study of leprosy patients.     

Maffucci syndrome with the spindle cell hemangiomas in the mucosa of the lower lip: a rare case report and literature review

The presence of non‐cutaneous vascular lesions in the syndrome of multiple enchondromas and subcutaneous hemangiomas, also named Maffucci syndrome, is exceedingly rare. Until now, non‐cutaneous vascular lesions have been described in nine patients, while only three cases were present in the oral cavity; they were found in the tongue in two patients and in the lower lip in one patient. Herein, we report the second case of vascular lesions localized in the mucosa of lower lip in a patient with Maffucci syndrome. Histopathologic examination showed spindle cell hemangioma.     

Oral lesion in leprosy: borderline tuberculoid diagnosis based on detection of Mycobacterium leprae DNA by qPCR

Oral lesions are rarely reported in paucibacillary forms of leprosy. We report here a case with an erythematous hyposensitive lesion in the palate and no skin lesions. In addition to routine tests, biopsies of the lesion in the palate and of clinically normal surrounding areas were performed and subjected to real-time PCR for detection of Mycobacterium leprae DNA. The biopsy of the oral lesion was positive for bacilli DNA, followed by positive serum anti-PGL-1 and Mitsuda test, but with negative histopathology. The patient was diagnosed with a borderline tuberculoid form. After multidrug therapy the lesion had significantly regressed and the bacilli DNA detection in the former lesion was negative. The bacilli DNA detection in an oral lesion by real-time PCR not only improved leprosy diagnosis, but also helped in the classification of clinical form, and in the establishment of the appropriate therapeutic regime.     

A clinico-pathological study of multidrug regimen in paucibacillary leprosy

Preliminary results of a clinical trial in one hundred untreated paucibacillary leprosy cases with multidrug therapy (MDT) as per WHO recommendation are presented. Out of 100 fresh cases studied 18 had indeterminate, 35 tuberculoid and 47 cases had borderline tuberculoid leprosy. All were given MDT consisting of rifampicin 600 mg once a month and dapsone 100 mg daily for six months. At the end of six months all the cases were evaluated clinically and histopathological examination of lesions were studied. The lesions were still active in 35% of patient clinically and 47% histologically. Complete histological resolution have come across only in 4 cases suffering from indeterminate leprosy. Altogether 65% cases receiving MDT have shown marked improvement to total inactivation. Histologically, lymphocytic infiltration still persisted in 90% of slides examined and nerve infiltration were still present in 64% of cases at the end of six months receiving MDT.     

Comparative study of the cutaneous sensation of leprosy-suspected lesions using Semmes-Weinstein monofilaments and quantitative thermal testing

The objective of the present study was to compare the warm cold perception thresholds (WPT), cold perception thresholds (CPT) and the warm and cold perception interval (WCPI) determined in our previous study with the touch-pressure thresholds, in leprosy-suspected skin lesions ('patch'). Thermal testing was conducted using a thermal sensory analyser TSA-2001 (Medoc Ltd., Israel) and the method of levels. The touch-pressure thresholds were measured using Semmes-Weinstein monofilament (SWM) of 0-05 g, 0.2 g, 2 g, 4 g, 10 g and 300 g. A cross-sectional study of 112 patients presenting with leprosy-suspected skin lesions, with no clinical evidence of peripheral nerve damage, was conducted. Leprosy diagnoses were based on clinical dermato-neurological examinations. One-hundred-and-eight subjects (45 males, 63 females; average age 37.7 years) completed the tests: 82 were positively diagnosed with leprosy and 26 with diseases of different aetiologies. The SWM test showed a sensitivity of 81.7% and a specificity of 96.1%, while the warm and cold perception thresholds presented sensitivity of 90.2% and 92-2%, respectively (both with 100% specificity). In leprosy patients, lesions that exhibited pressure thresholds of 0.05 g typically showed significantly different WPT, CPT and WCPI values when compared with skin lesions of different aetiologies. Within the leprosy group, the mean values of WPT, CPT and WCPI increased according to the increase in touch-pressure thresholds. Some of the patients exhibiting leprosy lesions with touch-pressure thresholds of 0-05 and 0-2 g presented normal WPT or CPT values. However, all patients with SWM equal or above 2.0 g presented altered WPT and CPT. All patients with leprosy, including those that exhibited pressure thresholds of 0.05 g, presented altered WCPI in the skin lesions. Despite a higher sensitivity to thermal tests, the SWM has adequate validity as a screening tool in the diagnosis of cutaneous forms of leprosy and in the selection of patients who should be submitted to a more detailed examination.