Leprosy in the Colombian island of Providencia / [Lepra en la isla colombiana de Providencia]

San Andrés and Providencia are Colombian islands in the Caribbean Sea. San Andrés has 68,283 inhabitants and has registered cases of leprosy in immigrants from continental Colombia. Providencia has 5,037 inhabitants and historically health programs did not have records of the disease, but in 2009 two cases of multibacillary histoid leprosy were confirmed and, subsequently, another two, which represents a prevalence of 8 cases per 10,000 inhabitants and places the island as a hyperendemic site for leprosy. Initially, a 14-year-old girl with histoid leprosy was diagnosed and, exploring this case, her father was diagnosed with the same clinical form of leprosy. Recently, a new intrafamilial patient with multibacillary leprosy and an extrafamilial case of a girl with undetermined leprosy were detected. The objective of this study was to present to the scientific community and the public health officers these clinical cases and to draw the attention of the sanitary authorities on the necessity of establishing continuous programs of leprosy epidemiological surveillance on the island using the new tools available in the Programa de Control de la Lepra (Leprosy Control Program).

San Andrés y Providencia son islas colombianas en el mar de las Antillas. San Andrés tiene 68.283 habitantes y allí se han registrado casos de lepra en inmigrantes provenientes del interior colombiano. Providencia tiene 5.037 habitantes e, históricamente, los programas de salud no tenían registros de la enfermedad; no obstante, en el 2009 se confirmaron dos casos de lepra multibacilar histioide y, posteriormente, otros dos, lo cual representa una prevalencia de 8 casos por 10.000 habitantes y la la convierte en un sitio hiperendémico para lepra. Inicialmente, se diagnosticó lepra histioide en una niña de 14 años y, durante su estudio, se encontró la misma forma clínica de la enfermedad en su padre. Recientemente, se detectó lepra multibacilar en otro miembro de la misma familia y, lepra indeterminada, en una niña de otro núcleo familiar. El objetivo de este trabajo fue presentar estos casos clínicos ante la comunidad científica y los entes de salud pública, y llamar la atención de las autoridades de salud sobre la necesidad de establecer programas de vigilancia epidemiológica continua en la isla, incorporando las nuevas herramientas disponibles en el Programa de Control de la Lepra.

Lepra en la isla colombiana de Providencia / Leprosy in the Colombian island of Providencia

San Andrés y Providencia son islas colombianas en el mar de las Antillas. San Andrés tiene 68.283 habitantes y allí se han registrado casos de lepra en inmigrantes provenientes del interior colombiano. Providencia tiene 5.037 habitantes e, históricamente, los programas de salud no tenían registros de la enfermedad; no obstante, en el 2009 se confirmaron dos casos de lepra multibacilar histioide y, posteriormente, otros dos, lo cual representa una prevalencia de 8 casos por 10.000 habitantes y la convierte en un sitio hiperendémico para lepra. Inicialmente, se diagnosticó lepra histioide en una niña de 14 años y, durante su estudio, se encontró la misma forma clínica de la enfermedad en su padre. Recientemente, se detectó lepra multibacilar en otro miembro de la misma familia y, lepra indeterminada, en una niña de otro núcleo familiar. El objetivo de este trabajo fue presentar estos casos clínicos ante la comunidad científica y los entes de salud pública, y llamar la atención de las autoridades de salud sobre la necesidad de establecer programas de vigilancia epidemiológica continua en la isla, incorporando las nuevas herramientas disponibles en el Programa de Control de la Lepra.

San Andrés and Providencia are Colombian islands in the Caribbean Sea. San Andrés has 68,283 inhabitants and has registered cases of leprosy in immigrants from continental Colombia. Providencia has 5,037 inhabitants and historically health programs did not have records of the disease, but in 2009 two cases of multibacillary histoid leprosy were confirmed and, subsequently, another two, which represents a prevalence of 8 cases per 10,000 inhabitants and places the island as a hyperendemic site for leprosy. Initially, a 14-year-old girl with histoid leprosy was diagnosed and, exploring this case, her father was diagnosed with the same clinical form of leprosy. Recently, a new intrafamilial patient with multibacillary leprosy and an extrafamilial case of a girl with undetermined leprosy were detected. The objective of this study was to present to the scientific community and the public health officers these clinical cases and to draw the attention of the sanitary authorities on the necessity of establishing continuous programs of leprosy epidemiological surveillance on the island using the new tools available in the Programa de Control de la Lepra (Leprosy Control Program).

Giant cells lepromatous leprosy. Diffuse dermatitis with exuberant foreign body giant cells in treated lepromatous leprosy / Células gigantes en lepra lepromatosa. Dermatitis difusa con células gigantes tipo cuerpo extraño exuberantes en lepra lepromatosa tratada

Patients with lepromatous leprosy that have received treatment for many years usually get follow up biopsies for persistent skin lesions or positive bacilloscopy even if the values are lower than in the initial bacilloscopy. We report the case of a 48-year old woman with long-standing lepromatous leprosy of 15 years of evolution, with a bacterial index of 4 in the direct smear and the initial skin biopsy. The patient was treated with multidrug therapy for 32 months although the treatment recommended by the World Health Organization (WHO) is only for 12 months. A skin biopsy was taken to determine if there was an active disease. We observed a diffuse dermal inflammation with numerous foreign body giant cells and vacuolated macrophages (Virchow´s cells). These cells contained granular acid-fast material that was also positive with immunohistochemistry for BCG. There were fragmented bacilli and the BI was 2. These cells were also strongly positive for CD68. The biopsy was interpreted as a residual form of lepromatous leprosy that did not require further multidrug therapy. We have observed similar histological profiles in several cases. The lack of clinical data makes it a histological challenge. The accumulation of lipids in these giant cells is due to bacillary destruction and fusion of vacuolated macrophages. We discuss here the role of bacillary and host lipids in the pathogenesis of lepromatous leprosy. We concluded that there was no need to extend the 12-month multidrug therapy recommended by WHO.

Los pacientes con lepra lepromatosa (LL) que han recibido tratamiento durante años, usualmente tienen seguimiento con biopsias de piel para lesiones persistentes o con baciloscopia positiva, con valores menores a los iniciales. Presentamos una mujer de 48 años con LL de 15 años de evolución, con índice bacilar (IB) 4 en el extendido directo y en la biopsia, que recibió terapia multidroga durante 32 meses, aunque el tratamiento recomendado por la Organización mundial de la salud (OMS) es de 12 meses. Se tomó una biopsia de piel para determinar si la enfermedad estaba activa. Se observó inflamación dérmica difusa con numerosas células gigantes tipo cuerpo extraño y macrófagos vacuolados (células de Virchow). Estas células, CD68 positivas, contenían material granular ácido-alcohol resistente, positivo con inmunohistoquímica para BCG. Se encontraron bacilos fragmentados y el IB fue de 2. Se interpretó como una forma residual de LL y que la paciente no requería MDT adicional. Este perfil histológico lo hemos observado en casos similares. Sin datos clínicos estas biopsias son un reto diagnóstico. La acumulación de lípidos en estas células gigantes se debe a la destrucción bacilar y a la fusión de macrófagos vacuolados. Revisamos el papel de los lípidos del bacilo y del huésped en la patogénesis de la LL. En estos casos no es necesario extender los 12 meses de MDT recomendados por la OMS. En el seguimiento de los pacientes se recomienda contar con los hallazgos clínicos, la baciloscopia, la biopsia anual de piel y los títulos IgM anti-glicolípido fenólico.

Células gigantes en lepra lepromatosa: dermatitis difusa con células gigantes exuberantes de tipo cuerpo extraño en lepra lepromatosa tratada / Giant cells lepromatous leprosy: Diffuse dermatitis with exuberant foreign body giant cells in treated lepromatous leprosy

Resumen Los pacientes con lepra lepromatosa que han recibido tratamiento durante años, usualmente requieren seguimiento con biopsias de piel para detectar lesiones persistentes o si la baciloscopia es positiva, incluso si los valores son menores que los iniciales. Se presenta el caso de una mujer de 48 años de edad con lepra lepromatosa de 15 años de evolución, índice bacilar de 4 en el extendido directo y en la biopsia, que recibió tratamiento con múltiples medicamentos durante 32 meses, aunque lo recomendado por la Organización Mundial de la Salud (OMS) es una duración de 12 meses. Se tomó una biopsia de piel para determinar si la enfermedad estaba activa. Se observó inflamación dérmica difusa con numerosas células gigantes de tipo cuerpo extraño y macrófagos vacuolados (células de Virchow). Estas células, CD68 positivas, contenían material granular ácido-alcohol resistente positivo con inmunohistoquímica para BCG. Se encontraron bacilos fragmentados y el índice bacilar fue de 2. Se interpretó como una forma residual de lepra lepromatosa y se concluyó que la paciente no requería prolongar el tratamiento con múltiples medicamentos. Este perfil histológico se ha observado en casos similares, pero sin datos clínicos estas biopsias representan un reto diagnóstico. La acumulación de lípidos en estas células gigantes se debe a la destrucción bacilar y a la fusión de macrófagos vacuolados. Se revisó el papel de los lípidos del bacilo y del huésped en la patogenia de la lepra lepromatosa. En estos casos, no es necesario extender los 12 meses de tratamiento con múltiples medicamentos recomendados por la OMS. En el seguimiento de los pacientes, se recomienda contar con los hallazgos clínicos, la baciloscopia, la biopsia anual de piel y los títulos IgM antiglucolípido fenólico.

Abstract Patients with lepromatous leprosy that have received treatment for many years usually get follow up biopsies for persistent skin lesions or positive bacilloscopy even if the values are lower than in the initial bacilloscopy. We report the case of a 48-year old woman with long-standing lepromatous leprosy of 15 years of evolution, with a bacterial index of 4 in the direct smear and the initial skin biopsy. The patient was treated with multidrug therapy for 32 months although the treatment recommended by the World Health Organization (WHO) is only for 12 months. A skin biopsy was taken to determine if there was an active disease. We observed a diffuse dermal inflammation with numerous foreign body giant cells and vacuolated macrophages (Virchow´s cells). These cells contained granular acid-fast material that was also positive with immunohistochemistry for BCG. There were fragmented bacilli and the BI was 2. These cells were also strongly positive for CD68. The biopsy was interpreted as a residual form of lepromatous leprosy that did not require further multidrug therapy. We have observed similar histological profiles in several cases. The lack of clinical data makes it a histological challenge. The accumulation of lipids in these giant cells is due to bacillary destruction and fusion of vacuolated macrophages. We discuss here the role of bacillary and host lipids in the pathogenesis of lepromatous leprosy. We concluded that there was no need to extend the 12-month multidrug therapy recommended by WHO. Clinical findings, bacilloscopy, annual skin biopsy, and anti-phenolic glycolipid-I IgM titers are recommended procedures for the follow-up of these patients.

La Lepra: una enfermedad vigente / Leprosy: an ongoing disease

El libro resalta que la lepra continúa siendo una enfermedad presente en Colombia y que aún constituye un problema de salud pública importante por los costos sociales, económicos y de sufrimiento humano que conlleva. Sabiendo que la literatura sobre el tema es escasa en nuestro medio, este libro surge como una herramienta de consulta creada para médicos y otros profesionales de salud, con la certeza de que es preciso mejorar la oportunidad del diagnóstico. Siendo fundamental que, durante su proceso formativo, todos los profesionales de la salud adquieran conocimientos sobre dicha enfermedad, que cada día se hace más visible por sus secuelas y diagnóstico tardío.

The book highlights the fact that leprosy continues to be a disease present in Colombia and that it is still a major public health problem due to the social, economic and human suffering costs it entails. Knowing that the literature on the subject is scarce in our country, this book is intended as a reference tool for doctors and other health professionals, in the knowledge that it is necessary to improve the timeliness of diagnosis. It is essential that, during their training process, all health professionals acquire knowledge about this disease, which is becoming more and more visible every day due to its sequelae and late diagnosis.

Lepra neural pura de 18 años de evolución / 18 years of pure neuritic leprosy

La lepra puede presentar dificultades diagnósticas, especialmente en la forma neural primaria. Un hombre de 38 años, exsoldado y trabajador rural, presentó durante más de 10 años hiperestesia supraclavicular y supraescapular izquierdas y anestesia progresiva escapular y en guante de la mano y brazo izquierdos, con reabsorción ósea de las falanges distales del primero y segundo dedos de la misma mano, cambios que le dificultaban realizar sus labores; no tenía lesiones cutáneas de lepra. El nervio cubital izquierdo presentaba engrosamiento epitroclear, por lo que se sospechó lepra neural pura. Las baciloscopias de moco, pabellones auriculares y codos fueron negativas, al igual que los anticuerpos IgM contra el glicolípido fenólico 1; dos biopsias de piel de zonas anestésicas fueron normales también. La electromiografía evidenció disminución notoria de los potenciales sensitivos de los nervios cubital, radial y mediano izquierdos, lo cual favoreció el diagnóstico de lepra neural primaria. Se inició tratamiento para lepra multibacilar por tener afección de más de un tronco nervioso, dos meses después comenzó a presentar mejoría notoria de los síntomas y logró reanudar sus actividades comunes y laborales. La lepra neural primaria cursa con zonas de hipoestesia y anestesia cutánea y engrosamiento neural troncular, puede permanecer sin diagnóstico durante años. La electromiografía, que demuestra disminución de los potenciales sensitivos, es una ayuda diagnóstica considerable, mientras la biopsia de piel anestésica ayuda al diagnóstico solo en la tercera parte de los casos. La sospecha clínica razonable es suficiente para iniciar tratamiento antileproso, cuando no se dispone de otros medios diagnósticos.

Leprosy can be difficult to diagnose, in particular the pure neuritic leprosy type. A 38-year-old male, former soldier and rural worker, presented with a 10-year history of supraclavicular and suprascapular hyperesthesia of the left side and progressive scapular left arm anesthesia, including the hand, associated with bone resorption in distal phalanges of the first and second finger. No typical leprosy skin changes were present. An examination of the skin revealed epitrochlear thickening of the left side cubital nerve, therefore primary neural leprosy was suspected. Skin smears from the routine sites were normal, as were two skin biopsies and the serologic assay specific for M leprae to detect phenolic glycolipid-1. Electromyography revealed an important reduction in the sensory action potential of the ulnar, radial and medial nerves, which favored the primary neural leprosy diagnosis. After two months of multibacillar leprosy treatment, the symptoms started to disappear and the patient could recommence his old job. Pure neuritic leprosy presents with skin areas of hypesthesia and anesthaesia associated with nerve thickening. It is a disease that is commonly misdiagnosed for several years before the correct diagnosis is made and effective treatment is started. Electromyography can be a helpful tool in the diagnosis, typically showing reduced sensory action potential, while a biopsy of anesthetic skin is only helpful in one-third of cases. Reasonable clinical suspicion is sufficient to initiate antileprosy treatment when no other diagnostic methods are available.

Perineural and intraneural cutaneous granulomas in granulomatous mycosis fungoides mimicking tuberculoid leprosy.

BACKGROUND: Histological similarities between granulomas and granulomatous mycosis fungoides (GMF) may lead to misdiagnoses of sarcoidosis or leprosy. METHODS: This report presents four patients with GMF in whom skin biopsies showed perineural and intraneural granulomas that were confused with tuberculoid leprosy granulomas. RESULTS: Patient 1 presented with erythematous plaques and bulky nodules. Biopsy findings suggested cutaneous sarcoidosis. Tumor resection showed granulomatous infiltrate extending to the fascia and skeletal muscle. Clinicopathological correlations permitted a diagnosis of GMF. Patient 2 presented with erythematous plaques. Skin biopsies had indicated sarcoidosis. Resection of a thigh nodule excluded leprosy, and GMF was diagnosed. Patient 3 presented with scaly, hyperpigmented plaques. Biopsy showed diffuse granulomatous inflammation with epithelioid and giant cells, abundant lymphocytes, and some eosinophils, and indicated GMF. Patient 4 presented with pruritic, erythematous plaques. Biopsy of an indurated mammary plaque initially indicated sarcoid granulomatous inflammation. Biopsy review suggested GMF. CONCLUSIONS: This study highlights both the diagnosis of GMF, and granulomatous cutaneous nerve injury in GMF and its possible confusion with leprosy granulomas. The histological diagnosis of GMF includes: (i) a granulomatous infiltrate rich in giant cells, emperipolesis, histiocytic cells, and scattered eosinophils, which may reach the fascia and muscle; (ii) the absence of elastic fibers or their phagocytosis by giant cells; and (iii) lymphocytes that may show atypia and epidermotropism. Deep biopsies reveal GMF diagnostic changes and, in conjunction with clinicopathological correlations, exclude a diagnosis of leprosy and support one of GMF, thus facilitating its appropriate management.

Histoid leprosy with giant lesions of fingers and toes.

This work was conducted at the Facultad de Medicina, Universidad de La Sabana, and at the Facultad de Medicina, Universidad Surcolombiana. Histoid leprosy, a clinical and histological variant of multibacillary leprosy, may offer a challenging diagnosis even for experts. An 83-year-old woman presented with papular, nodular and tumor-like lesions of 3 years of evolution, affecting fingers, toes, hands, thighs and knees, and wide superficial ulcers in her lower calves. Cutaneous lymphoma was suspected. A biopsy of a nodule of the knee showed a diffuse dermal infiltrate with microvacuolated histiocytes, moderate numbers of lymphocytes and plasma cells. Cutaneous lymphoma was suggested. Immunohistochemistry (IHC) showed prominent CD68-positive macrophages, as well as CD3, CD8 and CD20 positive cells. Additional sections suggested cutaneous leishmaniasis. New biopsies were sent with the clinical diagnoses of cutaneous lymphoma, Kaposi´s sarcoma or lepromatous leprosy, as the patient had madarosis. These biopsies showed atrophic epidermis, a thin Grenz zone and diffuse inflammation with fusiform cells and pale vacuolated macrophages. ZiehlNeelsen stain showed abundant solid phagocytized bacilli with no globii formation. Abundant bacilli were demonstrated in the first biopsy. Histoid leprosy was diagnosed. The patient received the WHO multidrug therapy with excellent results. We concluded that Ziehl Neelsen staining should be used in the presence of a diffuse dermal infiltrate with fusiform and vacuolated histiocytes, which suggests a tumor, and an IHC particularly rich in CD68-positive macrophages; this will reveal abundant bacilli if the lesion is leprosy. A good clinical pathological correlation is essential to establish a proper diagnosis and management of the patient.

Histoid leprosy with giant lesions of fingers and toes / Lepra histioide con lesiones gigantes de los dedos de manos y pies

This work was conducted at the Facultad de Medicina, Universidad de La Sabana, and at the Facultad de Medicina, Universidad Surcolombiana. Histoid leprosy, a clinical and histological variant of multibacillary leprosy, may offer a challenging diagnosis even for experts. An 83-year-old woman presented with papular, nodular and tumor-like lesions of 3 years of evolution, affecting fingers, toes, hands, thighs and knees, and wide superficial ulcers in her lower calves. Cutaneous lymphoma was suspected. A biopsy of a nodule of the knee showed a diffuse dermal infiltrate with microvacuolated histiocytes, moderate numbers of lymphocytes and plasma cells. Cutaneous lymphoma was suggested. Immunohistochemistry (IHC) showed prominent CD68-positive macrophages, as well as CD3, CD8 and CD20 positive cells. Additional sections suggested cutaneous leishmaniasis. New biopsies were sent with the clinical diagnoses of cutaneous lymphoma, Kaposi´s sarcoma or lepromatous leprosy, as the patient had madarosis. These biopsies showed atrophic epidermis, a thin Grenz zone and diffuse inflammation with fusiform cells and pale vacuolated macrophages. ZiehlNeelsen stain showed abundant solid phagocytized bacilli with no globii formation. Abundant bacilli were demonstrated in the first biopsy. Histoid leprosy was diagnosed. The patient received the WHO multidrug therapy with excellent results. We concluded that Ziehl Neelsen staining should be used in the presence of a diffuse dermal infiltrate with fusiform and vacuolated histiocytes, which suggests a tumor, and an IHC particularly rich in CD68-positive macrophages; this will reveal abundant bacilli if the lesion is leprosy. A good clinical pathological correlation is essential to establish a proper diagnosis and management of the patient.

La lepra histioide es una forma de lepra multibacilar de diagnóstico clínico e histológico difícil incluso para expertos. Una mujer de 83 años se presentó a consulta con pápulas, nódulos y tumores de tres años de evolución en los dedos de manos y pies, y en manos, muslos y rodillas, así como úlceras superficiales extensas en la porción inferior de las pantorrillas, ante lo cual se sospechó linfoma cutáneo. La biopsia de un nódulo de la rodilla mostró infiltrado dérmico difuso con histiocitos microvacuolados y algunos linfocitos y plasmocitos. Se sugirió la posibilidad de un linfoma cutáneo. La inmunohistoquímica demostró macrófagos prominentes positivos para CD68 y células CD3, CD8 y CD20. Con base en los cortes adicionales de la biopsia, se sugirió la presencia de leishmaniasis cutánea. Se tomaron nuevas biopsias con las sugerencias diagnósticas de linfoma cutáneo, sarcoma de Kaposi o lepra lepromatosa, pues la paciente presentaba madarosis. Estas mostraron epidermis atrófica, una delgada zona subepidérmica de colágeno denso y dermatitis difusa con células fusiformes y algunos macrófagos vacuolados. La coloración de Ziehl-Neelsen reveló la presencia de bacilos abundantes en los macrófagos, sin tendencia a formar globias. En la primera biopsia se demostraron abundantes bacilos. Se diagnosticó lepra histioide. La paciente recibió quimioterapia antileprosa (Organización Mundial de la Salud) con resultados excelentes. Se concluyó que un infiltrado dérmico difuso con histiocitos fusiformes y algunos vacuolados, que sugiere un tumor fusocelular, cuya inmunohistoquímica sea particularmente rica en células positivas para CD68, debe teñirse con Ziehl-Neelsen, lo que revelará abundantes bacilos si la lesión es de lepra. La adecuada correlación clínico-patológica es necesaria para establecer el diagnóstico y el manejo preciso del paciente.

Pure neuritic leprosy in patients from a high endemic region of Colombia.

Agua de Dios was a leprosarium for leprosy patients' obligatory isolation (1872-1961). Its leprosy incidence is the highest in Colombia (1.5-7/10000). Relapses are common. Government grant of US$ 200 per month subsidy is available to patients with disabilities. Spontaneous consultation with neural symptoms is frequent and simulation to get the subsidy has to be considered. We studied 36 subjects (2007-2009), with ages from 29-78, 19 of them men, with neural symptoms of 6 months to 20 years evolution. All had clinical examination, bacteriological examination, skin and nerve biopsies, electromyography (EMG), PCR for M. leprae, IgM anti-PGL1, and lepromin A. All but two are household contacts of leprosy patients. Symptoms were hypoesthesia of the hands and feet, and difficulty using hands with loss of muscular strength. None had skin lesions. Three had thickening of ulnar nerve. Lepromin was positive in all; bacteriology and biopsies were negative in all. The speed and amplitude of neural conduction were altered in 34 patients; two women had normal EMG and were considered to be feigning the disease; 21 were diagnosed as PNL by clinical, epidemiological and EMG findings; five of them had a positive PCR and one, high titers for IgM anti PGL1. Nine other subjects had diabetes and six carpal tunnel syndrome (CTS). Slow progression of disease, the lack of neural enlargement and the neural biopsies without inflammation suggest that most of these patients could have spontaneously cured PNL, as happens with other cases of paucibacillary leprosy. Diabetes and CTS are important differential diagnoses of PNL. Patients were treated with MDT and received the state subsidy.

[Leprosy and the testis]. / La lepra y el testículo.

INTRODUCTION: Damage of testicles is frequent in lepromatous leprosy and worsened by the presence of erythema nodosum leprosum. Objective. A patient is presented who developed lepromatous leprosy and erythema nodosum leprosum with major testicular compromise. MATERIAL AND METHODS: The 28-year-old male patient had lepromatous leprosy since age 22. During a polychemotherapy treatment for the lepromatous leprosy, he presented chronic erythema nodosum leprosum that affected both testicles; he did not respond to the conventional treatment. A left orchidectomy was performed to treat the persistent pain. RESULTS: The extracted testis evidenced the following: tubular atrophy, extensive fibrosis, cumulus of foamy macrophages without rods, focal Leydig cell hyperplasia, linfocitary and granulomatous arteritis and endarteritis of small and medium size vessels. These changes were also observed in the epididymis. Two years after the polychemoterapy and the orchidectomy, the patient exhibited azoospermy, normal total testosterone, slightly diminished free testosterone and elevated levels of luteinizing hormone and follicle-stimulating hormone. No loss of libido or sexual activity was reported. General concepts of erythema nodosum leprosum were reviewed, as well as the pathologic changes produced by leprosy in the testis. CONCLUSION: Lepromatous leprosy may lead to hypogonadism. This condition is recommended for inclusion in leprosy diagnostic programs in order to detect and treat the consequences of the possible hypogonadism.

La lepra y el testículo / Leprosy and the testis

Introducción. La afección testicular es frecuente en la lepra lepromatosa, daño que se incrementa cuando cursa con eritema nudoso leproso. Objetivo. Presentar un paciente con lepra lepromatosa y eritema nudoso leproso con grave compromiso testicular. Materiales y métodos. Se estudió un hombre de 28 años con lepra lepromatosa desde los 22, que durante la poliquimioterapia para la lepra presentó eritema nudoso leproso crónico que afectó ambos testículos y no respondió al manejo convencional. El dolor persistente obligó a practicar orquidectomía izquierda. Resultados. Este testículo presentaba atrofia tubular y fibrosis notorias, conglomerados de macrófagos espumosos, sin bacilos, hiperplasia focal de células de Leydig, endarteritis y arteritis linfocitaria y granulomatosa de vasos pequeños y medianos; estos cambios también estaban presentes en el epidídimo. Un estudio llevado a cabo dos años después de terminar su tratamiento y de la orquidectomía izquierda, demostró azoospermia, testosterona total normal, testosterona libre discretamente disminuida y hormonas lutropina (luteinizante) y folitropina (estimulante del folículo) elevadas. No había disminución de la libido ni de su actividad sexual. Se revisaron los conceptos generales sobre el eritema nudoso leproso y las alteraciones que la lepra produce en el testículo. Conclusión. La lepra lepromatosa puede conducir a hipogonadismo. Los programas de lepra deben contemplar esta complicación para corregir y evitar sus secuelas.

Introduction. Damage of testicles is frequent in lepromatous leprosy and worsened by the presence of erythema nodosum leprosum. Objective. A patient is presented who developed lepromatous leprosy and erythema nodosum leprosum with major testicular compromise. Material and methods. The 28-year-old male patient had lepromatous leprosy since age 22. During a polychemotherapy treatment for the lepromatous leprosy, he presented chronic erythema nodosum leprosum that affected both testicles; he did not respond to the conventional treatment. A left orchidectomy was performed to treat the persistent pain. Results. The extracted testis evidenced the following: tubular atrophy, extensive fibrosis, cumulus of foamy macrophages without rods, focal Leydig cell hyperplasia, linfocitary and granulomatous arteritis and endarteritis of small and medium size vessels. These changes were also observed in the epididymis. Two years after the polychemoterapy and the orchidectomy, the patient exhibited azoospermy, normal total testosterone, slightly diminished free testosterone and elevated levels of luteinizing hormone and follicle-stimulating hormone. No loss of libido or sexual activity was reported. General concepts of erythema nodosum leprosum were reviewed, as well as the pathologic changes produced by leprosy in the testis. Conclusion. Lepromatous leprosy may lead to hypogonadism. This condition is recommended for inclusion in leprosy diagnostic programs in order to detect and treat the consequences of the possible hypogonadism.

Lepra neural primaria: definición y criterios de manejo / Primary neural leprosy: definition and management criteria

La lepra neural pura, o primaria, es una neuropatía periférica. Es una forma clínica de la lepra en la cual no hay lesiones cutáneas ni historia de haberlas tenido. La afección principal ocurre en los troncos nerviosos que, usualmente, están engrosados y en cuyo trayecto se presenta hipoestesia o anestesia, y si el nervio es mixto, atrofia y pérdida funcional de los músculos inervados. Los nervios más comúnmente afectados son el cubital, el ciático poplíteo externo, el auricular y el mediano. El diagnóstico clínico es difícil y la enfermedad puede pasar desapercibida durante años, con daño neural cada vez con mayor discapacidad. El tener criterios clínicos, epidemiológicos, histopatológicos, inmunológicos, biomoleculares y electromiográficos sobre la enfermedad es de gran ayuda para su diagnóstico preciso. En este artículo, basado en nuestra experiencia, en la revisión de literatura sobre el tema y en la atención de pacientes en el Sanatorio de Agua de Dios (Cundinamarca), presentamos aspectos que cubren estas áreas del conocimiento de la lepra neural, los cuales son útiles para su manejo y accesibles de ser implementados en el Programa Nacional de Control de la Lepra.

[Persistent type 2 lepra reaction (erythema nodosum) and clofazimine-induced lethal enteropathy]. / Eritema nudoso leproso persistente y enteropatía letal por clofazimina.

INTRODUCTION: Clofazimine enterophathy is a serious complication of clofazimine when used at high doses for treatment of type 2 lepra or or erythema nodosum leprosum. Objective. A woman is presented who had a delayed diagnosis of leprosy, persistent type 2 lepra reaction and lethal clofazimine enteropathy. MATERIALS AND METHODS: A 31-year-old woman presented leprosy symptoms over a 16-year period without medical diagnosis of her disease. During this period, type 2 lepra episodes occurred, but were not accurately diagnosed. These episodes became more severe during her second pregnancy. The patient and her family were interviewed, and her clinical history reviewed. RESULTS: After twelve years of medical consults, lepromatous leprosy was diagnosed, based on perforation of her nasal septum, with a bacterial index of 5. Her husband and a 12-year-old daughter have leprosy symptoms. During multidrug therapy, she presented with repeated type 2 lepra reaction episodes for which she received daily clofazimine 400 mg doses. Two months after this treatment, severe and frequent episodes of intense abdominal pain began to occur. These persisted for more than a year and were managed with in-hospital administration of several classes of painkillers and antispasmodic medication, including morphine. She also presented with sporadic diarrhea, constipation, nausea, weight loss and mesenteric adenopathies. She died finally due to this intestinal condition. No autopsy was performed. CONCLUSIONS: The patient's clinical presentation suggested a clofazimine-induced lethal enteropathy, a complication not previously seen in Colombia. This connection was not recognized by the medical officers that treated the patient.

Eritema nudoso leproso persistente y enteropatía letal por clofazimina / Persistent type 2 lepra reaction and clofazimine-induced lethal enteropaty

Introducción. La enteropatía por clofazimina es una complicación grave de este fármaco, cuando se usa a dosis altas para la reacción leprosa tipo 2 y otras enfermedades. Objetivo. Presentar una mujer de 31 años con síntomas de lepra, incluidos episodios de eritema nudoso leproso, agravados durante el embarazo, sin diagnóstico médico preciso. Relatar la evolución de su enteropatía letal por clofazimina.Materiales y métodos. Entrevista con la paciente y sus familiares, revisión de la historia clínica y de la literatura pertinente. Resultados. La paciente presentó lesiones cutáneas anestésicas y varios episodios de eritema nudoso, agravados durante sus embarazos. Luego de epistaxis repetidas y perforación del tabique nasal, se diagnóstico lepra lepromatosa, 12 años después de numerosas consultas médicas. Su esposo y su hija de 12 años presentaron signos de lepra para la cual se trataron. La paciente tuvo episodios de reacción tipo 2 durante la poliquimioterapia, para los cuales recibió 400 mg diarios de clofazimina. A los dos meses de este tratamiento comenzó a presentar dolor abdominal persistente durante más de un año, muy serio y episódicamente exacerbado, manejado con analgésicos y antiespasmódicos, incluida la morfina. Tuvo, además, diarrea, estreñimiento, náuseas, pérdida de peso y adenopatías mesentéricas. Falleció sin diagnóstico de su afección intestinal. No se hizo autopsia. Conclusiones. La clínica final de la paciente sugiere que se trata de un caso de enteropatía letal por clofazimina, una complicación que no se había reconocido previamente en nuestros pacientes. Es necesario aumentar el conocimiento de la lepra entre los médicos.

Introduction. Clofazimine enterophathy is a serious complication of clofazimine when used at high doses for treatment of type 2 lepra or or erythema nodosum leprosum. Objective. A woman is presented who had a delayed diagnosis of leprosy, persistent type 2 lepra reaction and lethal clofazimine enteropathy. Materials and methods. A 31-year-old woman presented leprosy symptoms over a 16-year period without medical diagnosis of her disease. During this period, type 2 lepra episodes occurred, but were not accurately diagnosed. These episodes became more severe during her second pregnancy. The patient and her family were interviewed, and her clinical history reviewed. Results. After twelve years of medical consults, lepromatous leprosy was diagnosed, based on perforation of her nasal septum, with a bacterial index of 5. Her husband and a 12-year-old daughter have leprosy symptoms. During multidrug therapy, she presented with repeated type 2 lepra reaction episodes for which she received daily clofazimine 400 mg doses. Two months after this treatment, severe and frequent episodes of intense abdominal pain began to occur. These persisted for more than a year and were managed with in-hospital administration of several classes of painkillers and antispasmodic medication, including morphine. She also presented with sporadic diarrhea, constipation, nausea, weight loss and mesenteric adenopathies. She died finally due to this intestinal condition. No autopsy was performed. Conclusions The patient’s clinical presentation suggested a clofazimine-induced lethal enteropathy, a complication not previously seen in Colombia. This connection was not recognized by the medical officers that treated the patient.

Lepra en el anciano / Leprosy among aged patients

Objetivos. Evaluar las características clínicas y epidemiológicas de los pacientes con lepra mayores de 60 años de edad, con diagnóstico histológico del Instituto Nacional de Salud de Colombia, durante un periodo de 13 años. Materiales y métodos. Se realizó una revisión retrospectiva de los pacientes con lepra que tuvieran una edad mayor de 60 años, cuyas biopsias fueron enviadas al Instituto Nacional de Salud para confirmación histológica entre enero de 1990 y diciembre del 2002. Resultados. Entre enero de 1990 y diciembre del 2002 se confirmó la lepra por histología en el Instituto Nacional de Salud en 2.462 pacientes, de los cuales, 433 (17,6(por ciento)) tenían una edad mayor de 60 años (rango de 60 a 94). El 69(por ciento) de los casos correspondió a hombres, con una relación hombre:mujer de 2:1. El 51(por ciento) de los pacientes pertenecía al polo lepromatoso de la enfermedad. Las características clínicas fueron similares a las conocidas en otros grupos etarios. El tiempo de evolución de los síntomas fue menor de un año en 48,8(por ciento) de los pacientes. En el 10(por ciento) se encontró un estado reactivo. Los contactos sólo se informaron en 7(por ciento). El 9(por ciento) se presentó como recaída de una lepra previamente tratada. Discusión. El 17,6(por ciento) de los pacientes con lepra evaluados en el Instituto Nacional de Salud tienen más de 60 años, la mayoría de ellos son hombres y pertenecen al polo lepromatoso de la enfermedad con características clínicas similares a las de otros grupos. Los pacientes ancianos podrían contribuir a la diseminación de la lepra en nuestro país.

[Study of rifampin and dapsone resistance in three patients with recurring leprosy]. / Estudio de resistencia a la rifampicina y la dapsona en tres pacientes con recurrencia de lepra.

OBJECTIVE: To detect the presence of rifampin- and dapsone-resistant strains of Mycobacterium leprae in three patients with recurring leprosy and clinically-suspected antimicrobial resistance through molecular techniques. METHODS: A retrospective, descriptive study was conducted of three multibacillary patients at the "Agua de Dios" Sanitarium in Cundinamarca, Colombia, that presented leprosy relapses that were documented by medical history, bacilloscopy, and biopsy. Biopsies were taken of the skin lesions and the bacteria were subject to DNA extraction and purification. Regions of the rpoB and folP1 genes associated with antimicrobial resistance were amplified and subjected to touch-down polymerase chain reaction and the amplified products were sequenced using the Sanger method. RESULTS: A punctual mutation was identified in nucleotide 1367 of the rpoB gene in two of the samples studied. This mutation was not found in the folP1 gene of any of the three patients. CONCLUSIONS: The mutation identified showed strains of rifampin-resistant M. leprae in two of the three patients with recurring leprosy. Mutations that indicate dapsone-resistance were not detected in any of the three patients.

Estudio de resistencia a la rifampicina y la dapsona en tres pacientes con recurrencia de lepra / Study of rifampin and dapsone resistance in three patients with recurring leprosy

OBJETIVO: Detectar la presencia de cepas de Mycobacterium leprae resistentes a la rifampicina y la dapsona en tres pacientes con recurrencia de lepra y sospecha clínica de resistencia antimicrobiana, mediante la aplicación de técnicas moleculares. MÉTODOS: Se realizó un estudio descriptivo retrospectivo en tres pacientes multibacilares del Sanatorio de Agua de Dios, Cundinamarca, Colombia, que habían presentado recidivas de lepra documentadas por su historia clínica, baciloscopia y biopsia. Se obtuvieron biopsias de lesiones cutáneas que se procesaron para la extracción y purificación del ADN bacilar. Se amplificaron regiones de los genes rpoB y folP1 asociadas con la resistencia antimicrobiana, mediante la reacción en cadena de la polimerasa "touch-down" y se secuenciaron los productos amplificados mediante el método de Sanger. RESULTADOS: Se detectó una mutación puntual en el nucleótido 1367 del gen rpoB en dos de las muestras estudiadas. No se encontró la mutación estudiada en el gen folP1 en ninguno de los tres pacientes. CONCLUSIONES: La mutación identificada demostró la presencia de bacilos de M. leprae resistentes a la rifampicina en dos de los tres pacientes estudiados con recurrencia de la enfermedad. No se detectó la mutación indicadora de resistencia a la dapsona en ninguno de los tres pacientes.

OBJECTIVE: To detect the presence of rifampin- and dapsone-resistant strains of Mycobacterium leprae in three patients with recurring leprosy and clinically-suspected antimicrobial resistance through molecular techniques. METHODS: A retrospective, descriptive study was conducted of three multibacillary patients at the "Agua de Dios" Sanitarium in Cundinamarca, Colombia, that presented leprosy relapses that were documented by medical history, bacilloscopy, and biopsy. Biopsies were taken of the skin lesions and the bacteria were subject to DNA extraction and purification. Regions of the rpoB and folP1 genes associated with antimicrobial resistance were amplified and subjected to touch-down polymerase chain reaction and the amplified products were sequenced using the Sanger method. RESULTS: A punctual mutation was identified in nucleotide 1367 of the rpoB gene in two of the samples studied. This mutation was not found in the folP1 gene of any of the three patients. CONCLUSIONS: The mutation identified showed strains of rifampin-resistant M. leprae in two of the three patients with recurring leprosy. Mutations that indicate dapsone-resistance were not detected in any of the three patients.

[Active search for leprosy and other skin diseases in school children from Agua de Dios, Colombia]. / Búsqueda Activa de Lepra y de Otras Enfermedades de la Piel en Escolares de Agua de Dios, Colombia.

OBJECTIVE: Actively searching for leprosy, other skin diseases and BCG vaccination scars amongst school children from Agua de Dios, the municipality having the highest prevalence of leprosy in Colombia. METHODS: A clinical examination of the children was carried out by nurses, interns, general practitioners and experts on leprosy. Skin smear tests and skin biopsies were performed when the clinical findings suggested leprosy. Anti-phenolic glycolipid antibodies in blood were determined in special cases. RESULTS: 86 % of the 2 844 school children were examined; 833 had skin diseases and 16 % of these required evaluation by specialists. Four new cases of paucibacillary leprosy, two indeterminate and two primary polyneuritic cases were found. Pediculosis capitis, pityriasis alba, tinea versicolor, hypopigmented nevus, insect bites and miliaria were frequently detected. BCG vaccination scars were absent in 387 children; following several logistical problems, they were vaccinated. Four children had signs of childhood abuse. An 11-year-old girl presented hypopigmented mycosis fungoides. All diseases and conditions found were treated. The community received information regarding the results, emphasising the importance of an early diagnosis of leprosy. CONCLUSIONS: The incidence of leprosy found (16/10,000) was 123 times higher than the rest of the country's incidence. It is advisable to continue clinical examinations in Agua de Dios and research into risk factors for acquiring leprosy.