RESUMEN
ABSTRACT: The human T-cell lymphotropic virus type 1 is a retrovirus that may cause severe diseases such as infective dermatitis associated with HTLV-1 (IDH) and adult T-cell leukemia/lymphoma (ATL). IDH is a chronic relapsing infected eczema of childhood, and ATL is a distinct type of peripheral T-cell leukemia/lymphoma, which is classified into the following types: smoldering, primary cutaneous tumoral, chronic, lymphoma, and acute. Progression of IDH to ATL during the course of IDH has been previously reported in 3 young patients, two of them from Bahia (Brazil). We present the case of a 22-year-old man who had IDH since childhood and developed ATL 18 months ago. The lymphoma lesions were superimposed on previously existing IDH lesions (forehead, axillae, umbilical area, and neck) or in areas generally affected by IDH (external genitalia, hypogastrium, groin, and eyelid). Cutaneous lesions in ATL are very frequent, but in this patient, besides infiltrated plaques and papules presented vesicles on the skin corresponding histologically to dilated Pautrier abscesses. Vesicular ATL is a rare condition. This case constitutes a very demonstrative example of the close correlation between IDH and ATL.
Asunto(s)
Eccema , Infecciones por HTLV-I , Virus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T del Adulto , Linfoma , Enfermedades Cutáneas Infecciosas , Adulto , Infecciones por HTLV-I/complicaciones , Humanos , Leucemia-Linfoma de Células T del Adulto/complicaciones , Masculino , Enfermedades Cutáneas Infecciosas/complicaciones , Adulto JovenRESUMEN
Background: Human T-cell lymphotropic virus type-1 (HTLV-1) may cause severe diseases such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and infective dermatitis associated with HTLV-1 (IDH). The clinical characteristics and progression of 25 early onset HAM/TSP associated or not to IDH were described. Methods: Following-up 37 IDH patients with neurological examinations, 54% developed HAM/TSP. To these cases were added 5 cases of juvenile HAM/TSP. The patients were HTLV-1+ and were submitted to dermatological and neurological examinations. Diagnosis of HAM/TSP was performed according to Osame et al (1990) and Castro-Costa et al (2006) criteria. Results: Twenty-one patients were classified as definite HAM/TSP by both criteria, 3 as probable HAM/TSP by Osame et al, and another as probable HAM/TSP according to Castro-Costa et al Median age at onset of neurological manifestations was 9 years for the IDH/HAM/TSP group and 16 years for the HAM/TSP group (P = .045). In 12 patients, the onset of neurological manifestations occurred when they were less than 10 years of age. In the group IDH/HAM/TSP, the neurological symptoms always begun during the period of activity of IDH. The progression of HAM/TSP evaluated in 17 cases was heterogeneous, and 3 had rapid progressive course. Conclusions: The juvenile HAM/TSP may occur very early and also presents marked female predominance. Progression of IDH to HAM/TSP before 19 years of age is frequent (54%). Rapid progressive form may also occur in early HAM/TSP. As juvenile IDH and HAM/TSP are due to vertical transmission through breastfeeding, it is very important to avoid this pathway of infection.
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Progresión de la Enfermedad , Infecciones por HTLV-I/complicaciones , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Transmisión Vertical de Enfermedad Infecciosa , Paraparesia Espástica Tropical/virología , Adolescente , Brasil , Lactancia Materna/efectos adversos , Niño , Preescolar , Dermatitis/virología , Femenino , Humanos , Masculino , Factores Sexuales , Factores de TiempoRESUMEN
Human T-cell lymphotropic virus type 1 (HTLV-1) is endemic in some regions and its vertical transmission occurs mainly through breastfeeding. About 10% of carriers develop associated diseases including HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP), adult T-cell leukemia/lymphoma (ATL) and infectious dermatitis associated with HTLV-1 (IDH). We searched for available case reports of early-onset HAM/TSP and ATL to evaluate demographic and disease aspects in infantile-juvenile patients. In the reviewed literature, 27 HAM/TSP and 31 ATL cases were found. In almost all of them, the most likely route of transmission was through breastfeeding. ATL is rarely reported, notwithstanding it may be underestimated because T-cell lymphomas are not investigated for HTLV-1 infection in this age group. IDH was frequently associated with HAM/TSP. The investigation of HTLV-1 infection in pregnant women is an important matter of public health and should be mandatory in endemic countries.
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Virus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T del Adulto/epidemiología , Paraparesia Espástica Tropical/epidemiología , Adolescente , Adulto , Niño , Femenino , Humanos , Transmisión Vertical de Enfermedad Infecciosa , Masculino , Embarazo , Adulto JovenAsunto(s)
Antibacterianos/uso terapéutico , Nocardiosis/diagnóstico , Actinomicosis/diagnóstico , Diagnóstico Diferencial , Quimioterapia Combinada , Hospitalización , Humanos , Masculino , Nocardiosis/tratamiento farmacológico , Recurrencia , Sulfametoxazol/uso terapéutico , Trimetoprim/uso terapéuticoRESUMEN
Acquired cutis laxa (ACL) is a rare connective tissue disorder that affects the skin elastic fibers, resulting in the loss of elasticity. In 50% of cases, this condition is associated with other diseases, particularly plasma-cell dyscrasias. This report describes a case of ACL with unusual clinical and histopathological characteristics. A 29-year-old man presented with diffuse erythematous plaques that had first appeared 5 months previously. Examination revealed multiple flaccid erythematous plaques on his trunk, neck, and skinfolds. Immunophenotyping of bone marrow aspirate revealed 7% of monoclonal plasma cells with lambda light chain expression. Skin biopsy histology revealed foci of interstitial granulomatous reaction. Weigert stain showed a loss of elastic fibers in the dermis, areas with thickened fibers and elastophagocytosis. Immunohistochemistry was positive for CD68. The cutaneous findings enabled an early diagnosis of IgG lambda monoclonal gammopathy to be made. Microscopic examination revealed an interstitial granulomatous reaction and severe alterations in the elastic fibers that varied in intensity in the different biopsies. Curiously, little has been mentioned in the literature regarding the presence of an interstitial granulomatous reaction in ACL. It is our belief that this reaction is secondary to the degenerative process of the elastic fibers.
Asunto(s)
Cutis Laxo/etiología , Granuloma/etiología , Inmunoglobulina G/análisis , Cadenas lambda de Inmunoglobulina/análisis , Paraproteinemias/complicaciones , Piel/patología , Adulto , Biomarcadores/análisis , Biopsia , Cutis Laxo/inmunología , Cutis Laxo/patología , Diagnóstico Precoz , Tejido Elástico/patología , Granuloma/inmunología , Granuloma/patología , Humanos , Inmunohistoquímica , Inmunofenotipificación , Masculino , Paraproteinemias/diagnóstico , Paraproteinemias/inmunología , Valor Predictivo de las Pruebas , Piel/inmunologíaRESUMEN
BACKGROUND: Adult T-cell leukemia/lymphoma (ATL) is a severe disease caused by HTLV-I. This paper describes the clinicopathological and immunohistochemical findings of 52 cases of ATL with skin involvement and investigates whether there is any relationship between median survival time (MST) and histological patterns, primary cutaneous involvement and CD8 positivity. MATERIAL AND METHODS: All cases were HTLV-I+ and HIV- and were clinically classified. HTLV-I proviral integration was investigated in atypical cases. Immunohistochemistry was performed using CD3, CD4, CD5, CD7, CD8, CD20, CD25, CD30 and CD45RO markers. Ki-67 was used to evaluate the proliferative index. RESULTS: Twenty-seven cases were primary, while 25 were secondary. Monoclonal viral integration was demonstrated in all atypical cases. Patterns resembling mycosis fungoides (MF) were found in 19 cases and anaplastic large-cell lymphoma (ALCL) in two cases. Fifteen cases had an atypical immunophenotype and expressed CD8. Primary cutaneous ATL had a longer MST (48 months) than the secondary cutaneous ATL (7 months) and the difference was statistically significant, but no statistically significant difference was found between the MST of CD8-positive and negative cases. CONCLUSIONS: It is important to differentiate between primary and secondary cutaneous ATL and classify the cases histologically in order to better evaluate the prognosis. The two forms of primary cutaneous ATL, primary cutaneous smoldering and primary cutaneous tumoral (PCT), should also be identified. The smoldering type presented a longer survival (58 months) and histological aspects suggestive of better prognosis in contrast to the PCT type that had a shorter survival (20 months) and histological characteristics suggestive of worse outcome.
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Antígenos CD8/análisis , Linfocitos T CD8-positivos/virología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Leucemia-Linfoma de Células T del Adulto/diagnóstico , Leucemia-Linfoma de Células T del Adulto/mortalidad , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/mortalidad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Antígenos CD/análisis , Linfocitos T CD8-positivos/inmunología , Linfocitos T CD8-positivos/patología , Proliferación Celular , Niño , Femenino , Humanos , Inmunohistoquímica , Inmunofenotipificación , Antígeno Ki-67/análisis , Leucemia-Linfoma de Células T del Adulto/inmunología , Leucemia-Linfoma de Células T del Adulto/patología , Leucemia-Linfoma de Células T del Adulto/virología , Masculino , Persona de Mediana Edad , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/virología , Análisis de Supervivencia , Adulto JovenRESUMEN
Adult T-cell leukemia/lymphoma (ATL) is an aggressive type of leukemia/lymphoma associated with the human T-cell lymphotropic virus (HTLV-I). We describe an adult male patient clinically and pathologically diagnosed as mycosis fungoides and treated with chemotherapy after which complete involution of the lesions occurred. The disease relapsed with confluent dyshidrosis-like vesicles on the palmoplantar regions, followed by disseminated vesiculopapules and associated lymphocytosis. A serological test performed at this time revealed HTLV-I infection, and a diagnosis of chronic ATL was made. Monoclonal integration of HTLV-I was detected in peripheral blood mononuclear cells by inverse long polymerase chain reaction. A skin biopsy revealed spongiosis, Pautrier abscesses, and intraepidermal vesicles with atypical lymphocytes and an infiltration of small and atypical CD4 lymphocytes in the superficial dermis. Proliferative index (Ki-67) was 70%. This is the first reported vesicular cutaneous ATL with confirmation of HTLV-I proviral integration. The delay that occurred in diagnosing ATL was due to the fact that mycosis fungoides and ATL may present the same clinical, histopathological, and immunohistochemical features.
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Eccema Dishidrótico/patología , Infecciones por HTLV-I/patología , Leucemia-Linfoma de Células T del Adulto/patología , Micosis Fungoide/patología , Neoplasias Cutáneas/patología , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida , Diabetes Mellitus , Diagnóstico Diferencial , Doxorrubicina , Eccema Dishidrótico/virología , Infecciones por HTLV-I/complicaciones , Virus Linfotrópico T Tipo 1 Humano , Humanos , Inmunohistoquímica , Interferón-alfa/uso terapéutico , Leucemia-Linfoma de Células T del Adulto/tratamiento farmacológico , Leucemia-Linfoma de Células T del Adulto/virología , Masculino , Micosis Fungoide/tratamiento farmacológico , Reacción en Cadena de la Polimerasa , Prednisona , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/virología , Vincristina , Zidovudina/uso terapéuticoRESUMEN
INTRODUCTION: Infective dermatitis associated with HTLV-1 (IDH) is a recurrent eczema which affects children vertically infected with HTLV-1. In Bahia, Brazil, we recently reported that 47% of IDH patients also develop juvenile HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), a progressive disabling disorder which is typically reported in adult HTLV-1 carriers. IDH may also predispose to adult T-cell leukemia/lymphoma, a neoplasm associated with HTLV-1. The factors relating to the development of HTLV-1-associated juvenile diseases have not yet been defined. HTLV-1 proviral load (PVL) is one of the main parameters related to the development of HTLV-1 associated diseases in adults. In the current study, we investigated the role of PVL in IDH and juvenile HAM/TSP. METHODOLOGY/PRINCIPAL FINDINGS: This is a cohort study that included fifty-nine HTLV-1 infected children and adolescents, comprising 16 asymptomatic carriers, 18 IDH patients, 20 patients with IDH and HAM/TSP (IDH/HAM/TSP) and five with HAM/TSP. These patients were followed-up for up to 14 years (median of 8 years). We found that PVL in IDH and IDH/HAM/TSP patients were similarly higher than PVL in juvenile asymptomatic carriers (p<0.0001). In those IDH patients who developed HAM/TSP during follow-up, PVL levels did not vary significantly. HAM/TSP development did not occur in those IDH patients who presented high levels of PVL. IDH remission was associated with an increase of PVL. Inter-individual differences in PVL were observed within all groups. However, intra-individual PVL did not fluctuate significantly during follow-up. CONCLUSIONS/SIGNIFICANCE: High PVL in IDH patients was not necessary indicative of progression to HAM/TSP. PVL did not decrease after IDH remission. The maintenance of high PVL after remission could favor early development of ATL. Therefore, IDH patients would have to be followed-up even after remission of IDH and for a long period of time.
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Infecciones por HTLV-I/patología , Infecciones por HTLV-I/virología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Provirus/aislamiento & purificación , Carga Viral , Adolescente , Brasil , Niño , Preescolar , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Humanos , MasculinoRESUMEN
We describe a patient with human T cell lymphotropic virus type 1 (HTLV-1)-associated infective dermatitis who developed HTLV-1-associated myelopathy/tropical spastic paraparesis and adult T cell leukemia/lymphoma at 16 years of age. Long inverse polymerase chain reaction was used to demonstrate monoclonal integration of proviral DNA in the lymphomatous skin lesion.
Asunto(s)
Dermatitis/virología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Leucemia-Linfoma de Células T del Adulto/virología , Paraparesia Espástica Tropical/virología , Niño , Dermatitis/líquido cefalorraquídeo , Femenino , Anticuerpos Anti-HTLV-I/líquido cefalorraquídeo , Virus Linfotrópico T Tipo 1 Humano/inmunología , Humanos , Leucemia-Linfoma de Células T del Adulto/líquido cefalorraquídeo , Paraparesia Espástica Tropical/líquido cefalorraquídeo , Reacción en Cadena de la PolimerasaRESUMEN
The purpose of this study was to evaluate whether subdivision of adult T-cell leukemia/lymphoma (ATL) on the basis of clinical types, skin involvement, histologic features, cell size, and proliferative index (PI) was clinically relevant. Skin lesions were present in 47 cases (67%). Five cases were classified as primary cutaneous tumoral (PCT) type not included in the Shimoyama classification and characterized by skin tumors and absence of systemic involvement, lymphocytosis, and hypercalcemia. Mortality was high (61/70 [87%]). The overall median survival time (MST) was 12 months. The following variables were adversely related to survival: acute, lymphoma, and PCT types; absence of skin lesions; large cells; and PI more than 18%. The longer MST observed in cases with skin lesions was probably due to prolonged survival of the smoldering type (58 months). The MST of the PCT type (21 months) was shorter than that of the smoldering type, confirming the importance of clearly defining these 2 types of ATL.
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Leucemia-Linfoma de Células T del Adulto/diagnóstico , Linfoma de Células T/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Brasil/epidemiología , Niño , Femenino , Humanos , Leucemia-Linfoma de Células T del Adulto/clasificación , Leucemia-Linfoma de Células T del Adulto/mortalidad , Linfoma de Células T/clasificación , Linfoma de Células T/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Piel/patología , Tasa de SupervivenciaRESUMEN
Adult T-cell leukemia/lymphoma (ATL) is caused by the human T-cell lymphotropic virus type 1 (HTLV-1) which is endemic in countries of Caribbean and Central and South America. We performed a systematic search and review to identify publications on ATL in these countries to verify if this disease was getting recognition in these regions as well as the characteristics of the observed cases. The median age of 49.4 years was lower than that referred to in Japan. According to our findings in most Brazilian states and in some other countries, ATL is not being recognized and should be strongly considered in the differential diagnosis of T-cell leukemias/lymphomas. Failure to identify these cases may be due to the unsystematic realization of serology for HTLV-1 and phenotypic identification of non-Hodgkin lymphomas that may result from lack of resources. Detection of ATL cases has been more feasible with cooperation from foreign research centers. A huge effort should be made to improve the surveillance system for ATL diagnosis in most of the South- and Central-American and Caribbean countries, and this attitude should be embraced by public organs to support health professionals in this important task.
Asunto(s)
Infecciones por HTLV-I/epidemiología , Virus Linfotrópico T Tipo 1 Humano/inmunología , Leucemia-Linfoma de Células T del Adulto/epidemiología , Linfoma no Hodgkin/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Región del Caribe/epidemiología , América Central/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , América del Sur/epidemiología , Adulto JovenRESUMEN
Hodgkin-like ATLL is a rare variant of adult T-cell leukemia/lymphoma (ATLL), a disease caused by human T-cell lymphotropic virus type-1 (HTLV-1). At admission, a 46-year-old female presented with lymphadenomegaly, lymphocytosis, slight elevation of LDH blood level, and acid-alcohol resistant bacilli in sputum and was being treated for pulmonary tuberculosis (Tb). She had lymphocytosis in the previous 20 months. Serology for HTLV-1 was positive. Lymph node was infiltrated by medium-sized lymphocytes with scattered Hodgkin and Reed-Sternberg-like cells CD30+, CS1-4+, and CD79a+. Background cells were CD4+ and CD25+. A clinical diagnosis of favorable chronic ATLL was given. She was treated with chemotherapy but later progressed to acute ATLL and ultimately died. Hodgkin-like ATLL should be considered in the histological differential diagnosis with Hodgkin lymphoma since treatment and prognosis of these diseases are distinct. It is also important to search for HTLV-1 infection in patients with unexplained prolonged lymphocytosis.
Asunto(s)
Infecciones por HTLV-I/patología , Enfermedad de Hodgkin/patología , Leucemia-Linfoma de Células T del Adulto/patología , Linfocitosis/patología , Biopsia , Ensayo de Inmunoadsorción Enzimática , Resultado Fatal , Femenino , Enfermedad de Hodgkin/virología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Humanos , Leucemia-Linfoma de Células T del Adulto/virología , Ganglios Linfáticos/patología , Linfocitosis/virología , Persona de Mediana EdadRESUMEN
Entomophthoramycoses are classified into subcutaneous, mucocutaneous, and primary visceral forms. The mucocutaneous form, also known as rhinoentomophthoramycosis, involves the mucosa and subcutaneous tissues of the nose and is caused by Conidiobolus coronatus (Entomophthora coronata). In this report, we describe the first case of mucocutaneous entomophthoramycosis acquired by introduction of the fungus through the conjunctival mucosa as a consequence of trauma involving contamination with soil. The patient was a 37-year-old man with no other complaints. The lesion was tumoral and extended into the ethmoidal and maxillary sinuses. The histopathologic appearance of the lesion was characteristic of this infection with a granulomatous process rich in eosinophils and with hyphae surrounded by an eosinophilic, periodic acid-Schiff stain-positive halo (Splendore-Hoeppli phenomenon). To the best of our knowledge, this case constitutes the first report of mucocutaneous entomophthoramycosis acquired by ocular contamination.
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Enfermedades de la Conjuntiva/fisiopatología , Cigomicosis/fisiopatología , Adulto , Enfermedades de la Conjuntiva/patología , Humanos , Masculino , Deformidades Adquiridas Nasales/tratamiento farmacológico , Deformidades Adquiridas Nasales/microbiología , Deformidades Adquiridas Nasales/cirugía , Cigomicosis/tratamiento farmacológicoRESUMEN
Infective dermatitis associated with HTLV-I (IDH) is a chronic, infected childhood eczema. Two adult-onset cases of IDH were studied, one of which was associated with HAM/TSP. The patients were submitted to dermatological, neurological and pathological examination. Immunohistochemical studies were made using CD3, CD4, CD8, CD20, CD79a, and CD57 antibodies. Cytotoxic granules were investigated using granzyme B, perforin, and TIA. The patients presented infected erythematous, scaly lesions with mild itching and a good response to sulfamethoxazole/ trimethoprim. A differential diagnosis with atopic dermatitis (AD) and seborrheic dermatitis (SD) was made, based on: the distinctive morphology and distribution of the lesions, presence of exudative and infected lesions, and mild pruritus. The inflammatory infiltrate was composed predominantly of CD8+ lymphocytes that did not present cytotoxic granules. We concluded that IDH can begin in adulthood and may be associated with HAM/TSP. The immunohistochemical findings were different from those observed in AD and SD.
Asunto(s)
Infecciones por HTLV-I/patología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Enfermedades Cutáneas Virales/patología , Adulto , Biopsia con Aguja , Brasil , Femenino , Estudios de Seguimiento , Infecciones por HTLV-I/tratamiento farmacológico , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Enfermedades Cutáneas Virales/tratamiento farmacológico , Resultado del Tratamiento , Combinación Trimetoprim y Sulfametoxazol/uso terapéuticoRESUMEN
BACKGROUND: Infective dermatitis associated with human T cell lymphotropic virus type I (HTLV-I) infection is a chronic, relapsing eczema of childhood. METHODS: Children, their mothers, and their siblings underwent serological testing for HTLV-I. Epidemiological data were collected from all seropositive children and their family members, and clinical and dermatological examinations were performed. Laboratory studies, including skin culture, and histopathological analyses were also performed. The diagnosis of infective dermatitis associated with HTLV-I (IDH) was made according to previously established criteria. RESULTS: All of the patients with cases that demonstrated clinical aspects of IDH were positive for HTLV-I. The median age of the children at the time of the first visit was 8.0 years (range, 2-14 years). The median duration of breastfeeding for 19 children was 22.5 months (range, 1-48 months). The lesions were erythematous, scaly, exudative, and crusted in all cases. The scalp, retroauricular areas, neck, and groin were the regions that were commonly affected. Cultures were positive for Staphylococcus aureus for 95% of the patients. The children were followed-up for a median of 3.0 years (range, 0.1-7 years), and 5 children developed HTLV-I-associated myelopathy/tropical spastic paraparesis. All of the children except 1 were treated with sulfamethoxazole-trimethoprim, and their lesions either improved greatly or completely disappeared. CONCLUSIONS: The present study demonstrates the severity of IDH in Bahia and confirms that its diagnosis is based almost exclusively on clinical aspects of the disease. Serological testing for HTLV-I and careful follow-up is recommended for all children with chronic, relapsing, severe eczema in regions where HTLV-I is endemic.
Asunto(s)
Dermatitis/virología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Adolescente , Adulto , Brasil/epidemiología , Niño , Preescolar , Dermatitis/tratamiento farmacológico , Dermatitis/epidemiología , Dermatitis/patología , Humanos , Lactante , Piel/patologíaRESUMEN
BACKGROUND: Human T cell lymphotropic virus type 1 (HTLV-1)-associated infective dermatitis (IDH) is a chronic and recurrent eczema occurring during childhood and adolescence. HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic myelopathy of adulthood, presenting with slowly progressive spastic paraparesis and sphincter dysfunction with mild sensory involvement. There are few reports describing an association between IDH and HAM/TSP. The objective of this study was to evaluate the occurrence of HAM/TSP in patients with IDH and in seropositive members of their families and to determine the blood levels of antibodies against HTLV-1 in patients with HAM/TSP. METHODS: Twenty patients with IDH and their seropositive mothers and siblings underwent clinical, neurological, and laboratory evaluations. The diagnosis of HAM/TSP was made in accordance with the World Health Organization criteria. RESULTS: Nine individuals had HAM/TSP (6 of the patients with IDH, 2 mothers, and 1 seropositive brother). In 3 families, > 1 individual had HAM/TSP. The serum antibody titers of the patients with HAM/TSP varied from 1 : 3.125 to 1 : 78.125. CONCLUSIONS: A strong association was observed between IDH and HAM/TSP. The familial clustering of both diseases suggests a genetic background. Serological screening for HTLV-1 in children with symptoms of myelopathy is essential in areas where HTLV-1 is endemic.
Asunto(s)
Dermatitis/virología , Infecciones por HTLV-I/complicaciones , Infecciones por HTLV-I/genética , Paraparesia Espástica Tropical/complicaciones , Paraparesia Espástica Tropical/genética , Enfermedades Cutáneas Virales/complicaciones , Enfermedades Cutáneas Virales/genética , Adolescente , Anticuerpos Antivirales/sangre , Anticuerpos Antivirales/líquido cefalorraquídeo , Líquido Cefalorraquídeo/virología , Niño , Análisis por Conglomerados , Dermatitis/genética , Familia , Femenino , Infecciones por HTLV-I/diagnóstico , Virus Linfotrópico T Tipo 1 Humano/inmunología , Virus Linfotrópico T Tipo 1 Humano/aislamiento & purificación , Humanos , Masculino , Examen NeurológicoAsunto(s)
Leucemia-Linfoma de Células T del Adulto/patología , Neoplasias Cutáneas/patología , Adulto , Antígenos CD , Separación Celular , Diagnóstico Precoz , Citometría de Flujo , Humanos , Leucemia-Linfoma de Células T del Adulto/fisiopatología , Linfocitos/patología , Masculino , Fenotipo , Neoplasias Cutáneas/fisiopatologíaRESUMEN
Infective dermatitis associated with HTLV-I (IDH) is a chronic, recurrent, exudative eczema occurring in childhood which is considered to be a risk factor for the development of lymphoma and HTLV-I-associated myelopathy/tropical spastic paraparesis. Skin biopsies from 19 patients with IDH were studied histologically and immunohistochemically using the following antibodies: anti-CD3, CD45RO, CD20, CD79a, CD4, CD8, CD56, CD57, TIA-1, granzyme-B, and perforin. A chronic dermatitis similar to atopic and seborrheic dermatitis was observed in 15 cases, whereas architectural aspects mimicking mycosis fungoides were observed in the remaining four. The infiltrate consisted predominantly of CD8+ lymphocytes and of CD57+ cells in the dermis and epidermis. TIA-1 and granzyme-B were expressed in 15/18 cases and 5/19 cases at the proportion of < or = 15% and < or = 3%, respectively. All cases were negative for perforin and CD56. Like other dermatites, histologically IDH may represent a benign simulator of mycosis fungoides. IDH shows a predominance of CD8+ cells and a low percentage of cells with cytotoxic granules, indicating that most CD8+ lymphocytes are not activated. These findings differ from the immunohistochemical pattern of atopic and seborrheic dermatitis, possibly representing additional means of differentiation between IDH and these dermatites. The distribution of CD57+ cells suggests that they play a role in the inflammatory process.