Aspects of Langerhans cells and TNF-α in the cutaneous immunity of anogenital warts

Abstract Background: Anogenital warts are the leading sexually transmitted infection in patients seeking care at specialized clinics. They may display a vast array of forms, according to the interaction of the virus with the host's immunity. Cellular immunity is the epithelium's main form of defense against the virus, involving an active participation of the Langerhans cells and pro-inflammatory cytokines such as TNF-α. Objective: To assess the epithelial immune response of anogenital warts in males, according to the number of lesions presented. Methods: This is a prospective, cross-sectional study carried out at the dermatology outpatient clinic in a tertiary hospital. We included male patients over 18 years of age without comorbidities who had anogenital condylomata and no previous treatments.In order to evaluate the local epithelial immunity, the lesions were quantified, then removed and employed in CD1a immunohistochemistry assays for assessing the morphometry and morphology of Langerhans cells; TNF-α; reaction was used for determining cytokine positivity in the epithelium. Results: 48 patients were included in the study. There was no statistically significant difference as to the number of Langerhans cells, in their morphology, or the presence of TNF-α. However, patients presenting with more Langerhans cells in the lesions had cells with a star-like and dendritic morphology, whereas in those with a lower cell count had cells with a rounded morphology and no dendrites (p < 0.001). Study limitations: Small number of patients analyzed. Conclusion: There was no difference in epithelial immunity between patients having few or many anogenital condyloma lesions as measured by the morphology and morphometry of Langerhans cells and TNF-α; positivity. Such an assessment employing immunity markers differing from the usual ones is expected to yield useful results.

Comparação da Quantidade de Células de Langerhans em Neoplasias Intraepiteliais Cervicais e Cervicites Crônicas / Comparison of the Quantity of Langerhans Cells in Cervical Intraepithelial Neoplasia and Chronic Cervicitis / Comparación de la Cantidad de Células de Langerhans en Neoplasias Intraepiteliales Cervicales y Cervicites Crónicas

Introdução: O câncer cervical é atribuído ao papilomavírus humano (HPV) cuja infecção, na maioria das vezes, sofre regressão espontânea. A menor porção de casos que evoluem para lesão precursora de baixo e alto graus e invasora pode ter relação com uma falha na atividade das células de Langerhans em eliminar o vírus. Objetivo: Determinar se há redução do número de células de Langerhans em colos uterinos acometidos por neoplasias intraepiteliais cervicais (NIC), graus I e III, comparado ao grupo controle (cervicites crônicas), por imuno-histoquímica, possibilitando correlacionar a ação do sistema imune com o desenvolvimento dessas lesões. Método: Foram analisados 40 casos de cervicite crônica, NIC I e III, com diagnóstico anatomopatológico entre janeiro de 2014 e dezembro de 2015, buscando-se comparar a quantidade de núcleos marcados positivamente como célula de Langerhans pela proteína S-100 por imuno-histoquímica, quantificando-os em áreas padronizadas. Resultados: Dos 40 casos avaliados, 17 foram cervicite crônica, 13 NIC I e 10 NIC III. Na análise comparativa do número de células em cada grupo a média, desvio-padrão e mediana foram maiores no grupo cervicite crônica e menores no grupo NIC III. O valor de p encontrado para a variação do número de células de Langerhans, entre os grupos, foi significativo (p=0,0442); mas, ao comparar os grupos de NIC com o controle, só o grupo NIC III teve variação significativa (p=0,0209). Conclusão:Há diminuição significativa do número de núcleos de células de Langerhans marcados em lesões do tipo NIC III em comparação a cervicites crônicas.

Introduction: Cervical cancer is attributed to human papillomavirus (HPV), whose infection mostly undergoes spontaneous regression. The smaller part of cases that evolve to low and high-grade lesions or invasive lesions may be related to failure of Langerhans cell activity to eliminate the virus. Objective: To determine if there is reduction of Langerhans' cells in cervix uterus affected by cervical intraepithelial neoplasms (CIN) grades I and III compared to control group (chronic cervicitis) by immunohistochemistry, granting the correlation of the immune system action with the development of these lesions. Method: It were analyzed 40 cases of chronic cervicitis, CIN I and III with anatomopathological diagnosis between January 2014 and December 2015, attempting to compare the amount of positively labeled Langerhans cells nuclei by S-100 protein by immunohistochemistry, quantifying them in standard areas. Results: Of the 40 evaluated cases, 17 were chronic cervicitis, 13 CIN I and 10 CIN III. The comparative analysis of the number of cells in each group showed that the mean, standard deviation and median number of Langerhans cells per area were higher in the chronic cervicitis group and lower in the CIN III group. The p value found in the variation of the Langerhans cells number among the groups was significant (p=0.0442). However, when comparing the CIN groups directly with the control group, only the CIN III group had a significant variation (p=0.0209). Conclusion: There is a significant decrease in the number of marked Langerhans cell nuclei in CIN III type lesions compared to chronic cervicitis.

Introducción: El cáncer cervical puede atribuirse al virus del papiloma humano (VPH) cuya infección a menudo sufre regresión espontánea. El menor número de casos que evolucionan a lesiones precursoras de bajo y alto grado o invasivas puede estar relacionado con una falla en la actividad de las células de Langerhans para eliminar el virus. Objetivo: Determinar si hay reducción del número de células de Langerhans en colos uterinos acometidos por neoplasias intraepiteliales cervicales (NIC) grados I y III comparado al grupo control (cervicitis crónicas), por medio de inmunohistoquímica, posibilitando correlacionar la acción del sistema inmune con estas lesiones. Método: Se analizaron 40 casos de cervicitis crónica, NIC I y III, con diagnóstico anatomopatológico entre enero de 2014 y diciembre de 2015, comparando la cantidad de núcleos marcados positivamente como célula de Langerhans por la proteína S-100 por inmuno-histoquímica, cuantificándolos. Resultados: De 40 casos, 17 fueron cervicitis crónica, 13 NIC I y 10 NIC III. En el análisis comparativo del número de células en cada grupo la media, desviación estándar y mediana fueron mayores en el grupo cervicite crónica y menores en el NIC III. El valor de p encontrado para la variación del número de células de Langerhans entre los grupos fue significativo (p=0,0442), pero al comparar los grupos de NIC con el control sólo el grupo NIC III tuvo variación significativa (p=0,0209). Conclusión: Hay disminución significativa del número de núcleos marcados de células de Langerhans en lesiones de tipo CIN III en comparación con cervicitis crónica.

Histiocitosis de células de Langerhans: el "camaleón" de la dermatopediatría / Langerhans cell histiocytosis

La Histiocitosis de Células de Langerhans (HCL) es una neoplasia mieloide de las células dendríticas Langerhans (CDL), caracterizada por acúmulos de células dendríticas mieloides CD207+. Corresponden a un espectro de enfermedades, desde sólo cutáneas a variantes multiorgánicas. El objetivo de este reporte es describir el caso clínico de un paciente pediátrico, con diagnóstico de Histiocitosis de Células de Langerhans, enfatizando el algoritmo clínico. Paciente masculino de 1 año y 5 meses, con antecedentes de otorrea persistente, refractaria a tratamiento, de un año de evolución. Consulta en policlínico de dermatología por "dermatitis severa" desde hace 1 año. Al examen físico se constatan lesiones tipo dermatitis seborreica generalizadas en tronco y cuero cabelludo, intertrigo fisurado, pápulas eritemato-costrosas plantares con petequias y pus en conducto auditivo externo bilateral. Presenta Hemoglobina 9,5 mg/dl, Hematocrito31,9%, leucocitos 12.400, linfocitos 33,3%, plaquetas 920.000, VHS 27. Subpoblaciones linfocitarias: CD3: 34,7%, C4: 22,7%, CD8: 9,7%, CD19:47,8%. HTLV negativo, VIH negativo. Acaro-test negativo. Dermatopatología: Denso infiltrado de células linfomonocíticas en dermis papilar, con ensanchamiento de estas y gran epidermotropismo, con abundante citoplasma eosinófilo con núcleos arriñonados, CD1a y langerina positivo. Recomendamos elevar la sospecha diagnóstica ante un cuadro de dermatitis seborreica generalizada que esta fuera del rango etario característico y en casos de dermatitis refractarias, donde a pesar de un adecuado tratamiento médico, el paciente persiste comprometido.

Langerhans Cell Histiocytosis (HCL) is a myeloid neoplasm of Langerhans dendritic cells (CDL), characterized by accumulations of myeloid dendritic cells CD207 +. They correspond to a spectrum of diseases, from cutaneous to multi-organ variants. The objective of this report is to describe the clinical case of a pediatric patient with diagnosis of, emphasizing the clinical algorithm. Male patient,1 year and 5 months old, with a history of refractory persistent otorrhea, consulted because of long term severe dermatitis. Physical examination revealed generalized seborrheic dermatitis lesions on the trunk and scalp, cleft intertrigo, plantar erythematous-crusted papules with petechiae, and pus in the external auditory canal. Laboratory findings showed: Hemoglobin 9.5 mg / dl, Hematocrit: 31.9%, leukocytes: 12,400, lymphocytes 33.3%, platelets: 920,000, HSV 27. Lymphocyte subpopulations: CD3: 34.7%, C4: 22.7%, CD8: 9.7%, CD19: 47.8%. HTLV negative, HIV negative. Scabies Negative. Dermatopathology: Dense infiltrate of lymphomonocytic cells in the papillary dermis with widening of the papilla and large epidermotropism, cells show abundant eosinophilic cytoplasm with "kidney nuclei", CD1a and langerin were positive. We recommend elevating the diagnostic suspicion in the face of a generalized seborrheic dermatitis that is outside the characteristic age range and in cases of refractory dermatitis, where the patient persists compromised.

The molecular fingerprint of human papillomavirus infection and its effect on the Langerhans cell population in squamous cell carcinomas of the genital skin.

Background: Information is scarce about the presence of molecular alterations related to human papillomavirus (HPV) infection in squamous cell carcinomas of the genital skin and about the effect of this infection in the number of Langerhans cells present in these tumors. Aims: To determine the presence of HPV in genital skin squamous cell carcinomas and to see the relationship between HPV infection and changes in the expression of Ki-67 antigen (Ki-67), p53 protein (p53), retinoblastoma protein (pRb) and E-cadherin and to alterations in Langerhans cell density, if any. Methods: A descriptive, comparative, retrospective and cross-sectional study was performed with all the cases diagnosed as squamous cell carcinomas of the genital skin at the Dermatopathology Service from 2001 to 2011. The diagnosis was verified by histopathological examination. The presence of HPV was examined using chromogenic in situ hybridization, and protein expression was studied via immunohistochemical analysis. Results: The 34 cases studied were verified as squamous cell carcinomas and 44.1% were HPV positive. The degree of expression of pRb was 17.50% ±14.11% (mean ± SD) in HPV-positive cases and 29.74% ±20.38% in HPV-negative cases (P = 0.0236). The degree of expression of Ki-67 was 47.67% ±30.64% in HPV-positive cases and 29.87% ±15.95% in HPV-negative cases (P = 0.0273). Conclusion: HPV infection was related to lower pRb expression and higher Ki-67 expression in comparison with HPV negative samples. We could not find a relationship between HPV infection and the degree of expression of p53 and E-cadherin or with Langerhans cell density.

Tissue immunostaining for factor XIIIa in dermal dendrocytes of pityriasis alba skin lesions

BACKGROUND: Pityriasis alba affects 1% of the world population and about 9.9% of the children in Brazil. However, its etiology remains uncertain. OBJECTIVE: The objective of the present study was to evaluate the immunoexpression of factor XIIIa in dermal dendrocytes of skin lesions of pityriasis alba. METHOD: Twenty patients with pityriasis alba and 20 patients with atopic dermatitis underwent biopsy. The dermal dendrocytes marked by factor XIIIa were counted by means of immunohistochemical analysis. RESULTS: The mean amount of dermal dendrocytes found in the patients with pityriasis alba was 2, whereas in the patients with atopic dermatitis it was 4, with a statistically significant difference between them. A cutoff point of 3 cells/square inch was established to differentiate pityriasis alba from atopic dermatitis, with 80% sensibility and 90% specificity. CONCLUSION: We believe that pityriasis alba and atopic dermatitis should be considered different clinical forms within the spectrum of atopic disease, in which sun radiation plays an important role by modulating the progression of the disease. .

Immunohistochemistry assessment of Langerhans cells and microvascular density in oral squamous cell carcinomas / Avaliação imuno-histoquímica de células de Langerhans e densidade microvascular em carcinoma oral de células escamosas

The aim of this study was to assess and correlate microvascular density (MVD) and the quantity of Langerhans cells (LC) present in oral squamous cell carcinoma (OSCC), well as the correlation between this microvascular density and number of Langerhans cells (LCs) with the intensity of the infiltrate, the histologic grading and staging, according to the TNM system. Twenty-three paraffin-embedded blocks of SCC lesions were analyzed using the immunohistochemical technique in which the two anti-CD1a and anti-CD207 markers were used to quantify the Langerhans cells and the CD34 marker to assess MVD. Immunostaining for CD1a, CD207 and CD34 was observed in 100% of the cases analyzed, showing a statistically significant association (p = 0.0001, Fisher’s test). No statistical correlation between MVD and LC or between immunostainings and histological grading of malignancy were found. However, immunostaining for CD1a and CD207 showed a statistically significant correlation (p value = 0.001, Spearman test) and a positive correlation was found between MVD and lymph node involvement. The LCs and MVD seem to involved in immunopathogenesis of oral carcinoma, although no statistically significant correlation was found between these two findings

O objetivo deste estudo foi avaliar e correlacionar a densidade microvascular (MVD) e a quantidade de células de Langerhans (LC) presente no carcinoma epidermoide de boca (CEB), bem como a correlação entre esta densidade microvascular e número das células de Langerhans (CL), com a intensidade do infiltrado, a classificação histológica e de teste, de acordo com o sistema TNM. Vinte e três blocos de parafina-encaixados de lesães SCC foram analisados utilizando a técnica de imuno-histoquímica em que os dois marcadores anti-CD1a e anti-CD207 foram usados para quantificar as células de Langerhans e o marcador CD34 para avaliar MVD. A imunocoloração para CD1a, CD207 e CD34 foi observada em 100% dos casos analisados, demonstrando uma associação estatisticamente significativa (p = 0,0001, teste de Fisher). Não houve correlação estatística entre MVD e LC ou entre imunomarcações e gradação histológica de malignidade foram encontrados. No entanto, a imunocoloração para CD1a e CD207 mostraram uma correlação estatisticamente significativa (p = 0,001, teste de Spearman) e foi encontrada uma correlação positiva entre MVD e comprometimento de linfonodos. O LCs e MVD parecem envolvidos em imunopatogênese de carcinoma oral, embora não foi encontrada correlação estatisticamente significativa entre estes dois resultados.

Evaluation of Langerhans cells counts comparing HIV-positive and negative anal squamous cell-carcinoma patients / Avaliação das células de Langerhans no carcinoma espinocelular do canal anal em pacientes HIV-positivo e negativo

PURPOSE: To investigate the differences in Langerhans cells (LCs) populations between HIV-positive and negative anal squamous cell carcinomas patients. METHODS: Twenty five patients (14 HIV-positive and 11 HIV-negative) were evaluated. Paraffin-block transversal thin sections from biopsies of anal squamous cell carcinomas (ASCC) were stained using the anti-CD1A antibody that identifies activated LCs. LCs counts were performed using histometry at 20 different sites, at baseline in the ASCC cases. These were then compared with LCs counts in anal canal specimens from HIV-negative and positive patients without ASCC (controls groups). RESULTS: In patients with ASCC, the LC count was greater among HIV-negative individuals than among HIV-positive individuals (p<0.05). The LC count was greater in the control HIV-negative group than in HIV-positive patients with ASCC (p<0.05). CONCLUSION: There was a lower amount of activated LCs in HIV-positive patients with anal squamous cell carcinomas than in HIV-negative patients, thereby suggesting worsening of the immune response.

OBJETIVO: Comparar a quantidade de células de Langerhans (CL) em pacientes portadores do carcinoma espinocelular (CEC) do canal anal HIV-positivo e negativo. MÉTODOS: Avaliamos 25 pacientes, sendo 11 HIV-negativo e 14 HIV-positivo portadores do CEC do canal anal. Realizamos estudo com a coloração imunoistoquímica anti-CD1A para avaliar as CL ativadas. Utilizamos as lâminas coradas e pelo método da histometria contamos em 20 campos diferentes as células coradas na camada basal da lâmina própria, onde era evidente a disseminação tumoral. Realizamos dois grupos controles compostos por pacientes submetidos à biopsia anal sem neoplasia (sete pacientes HIV-negativo e quatro HIV-positivo). Comparamos as contagens de CL. RESULTADOS: A quantidade de CL foi superior nos pacientes portadores do CEC do canal anal soronegativo para o HIV, em relação aos soropositivos (p<0,05). A quantidade de CL foi superior no grupo controle HIV-negativo em relação ao grupo composto por pacientes soropositivos portadores do CEC do canal anal (p<0,05). CONCLUSÃO: Houve aumento das células de Langerhans ativadas na área peritumoral dos pacientes soropositivos para o HIV, o que sugere diminuição da resposta imune local.

Langerhans cells in periodontal disease of HIV- and HIV+ patients undergoing highly active antiretroviral therapy

The aim of this study was to assess and compare quantitatively the presence of S100+ Langerhans cells (LC) by immunochemistry techniques in HIV+ and HIV- gingivitis and periodontitis subjects. Additionally, it aimed to evaluate the correlation among densities of these cells with CD4+ and CD8+ T cells, and viral load levels in HIV+ subjects, all using Highly Active Antiretroviral Therapy (HAART). The samples were allocated into four groups: 1) 15 subjects with moderate chronic periodontitis (MCP), HIV+; 2) 15 subjects with MCP, HIV-; 3) 10 subjects with gingivitis (G), HIV+; and 4) 10 subjects with G, HIV-. The S100+ cells were assessed in the pocket epithelium, gingival epithelium, and lamina propria. A statistically significant increase of total S100+ cells in HIV+ periodontitis subjects was observed in relation to HIV- periodontitis subjects. No increase of S100+ cells with increased inflammation was observed. No statistically significant correlation among S100+ cells and blood levels of CD4, CD8, and viral load was observed. In conclusion, the use of HAART can aid in achieving viral loads, and it is suggested that it may prevent the destruction of the LC.

Doença enxerto contra hospedeiro crônica em mucosa bucal: relação da concentração de células de Langerhans com a expressão da quimiocina CCL20 e de seu receptor CCR6 / Chronic graft versus host disease in the oral mucosa: concentration of Langerhans cells and its relationship with the chemokine CCL20 and its receptor CCR6

A doença enxerto contra hospedeiro (GVHD) é uma complicação comum nos pacientes submetidos ao transplante de células-tronco hematopoiéticas (TCTH), sendo considerada a maior causa de morbidade e mortalidade nesses pacientes. O principal objetivo do presente estudo foi relacionar a concentração de células de Langerhans em mucosa bucal de pacientes com GVHDc bucal com a expressão da quimiocina CCL20 e de seu receptor CCR6 no epitélio bucal, a fim de elucidar os mecanismos biológicos envolvidos no recrutamento das células de Langerhans na GVHDc. Foram selecionados fragmentos obtidos por biópsia de mucosa bucal de 60 pacientes onco-hematológicos e hematológicos submetidos previamente ao transplante de células tronco hematopoiéticas no Hospital Amaral Carvalho, Jaú SP, onde 30 pacientes desenvolveram GVHDc em mucosa bucal (Grupo 1) e 30 não desenvolveram GVHDc (Grupo 2). Amostras obtidas a partir de 30 biópsias de lesões não inflamatórias em mucosa bucal constituíram o Grupo Controle (Grupo 3). Cortes microscópicos foram avaliados em coloração de rotina Hematoxilina e Eosina, e submetidos à técnica imuno-histoquímica, utilizando-se anticorpos monoclonais anti-CD1a e anti-CCR6, e anticorpos policlonais anti-CCL20. As células de Langerhans CD1a+ foram quantificadas no epitélio da mucosa bucal, e os resultados demonstraram um maior número destas células nos pacientes com GVHDc quando comparados àqueles sem GVHDc e ao Grupo Controle (p<0,001). A análise da imunomarcação das moléculas CCR6 e CCL20 foi subjetiva com aplicação de escores. Quanto à molécula CCR6, houve maior expressão no Grupo 1 (p<0,001) em comparação aos outros Grupos; porém, quanto à expressão de CCL20, não houve diferença estatística entre os três Grupos (p=0,108). Estes resultados sugerem que o aumento das células de Langerhans, na doença enxerto contra hospedeiro crônica, em mucosa bucal, pode estar associado a maior expressão do receptor CCR6. Possivelmente, o maior recrutamento de células de...

The graft versus host disease (GVHD) is a common complication in patients undergoing hematopoietic stem cell transplantation (HSCT), and considered a major cause of morbidity and mortality in these patients. The main objective of this study was to compare the concentration of Langerhans cells in oral mucosa of patients with oral chronic GVHD (GVHDc) with the expression of the chemokine CCL20 and its receptor CCR6 in oral epithelium, in order to clarify the biological mechanisms involved in the recruitment of Langerhans cells in GVHDc. We selected 60 biopsies of oral mucosa from onco-hematological and hematological patients submitted to prior hematopoietic stem cell transplantation at Hospital Amaral Carvalho, Jaú - SP from which 30 patients developed GVHDc in the oral mucosa (Group 1) and 30 did not develop GVHDc (Group 2). The Control Group (Group 3) was obtained from 30 biopsies of non-inflammatory lesions of oral mucosa. Microscopic sections were evaluated in routine Hematoxylin and Eosin staining, and submitted to immunohistochemistry using anti-CD1a and anti-CCR6 monoclonal antibodies, and anti-CCL20 polyclonal antibody. The Langerhans cells (CD1a+) were quantified in the epithelium of the oral mucosa, and the results showed a greater number of these cells in patients with GVHDc compared to those without GVHDc and the Control Group (p<0.001). Analysis of immunostaining of molecules CCL20 and CCR6 were subjective with application of scores. The expression of CCR6 molecule was more significant in Group 1 (p<0.001) compared to other groups, but in relation to CCL20 expression, there was no statistical difference between the three groups (p=0.108). These results suggest that the increase of Langerhans cells in GVHDc affecting oral mucosa may be associated with increased expression of the receptor CCR6. We suggest that the increased recruitment of Langerhans cells to the oral mucosa in patients with transplanted bone marrow contributes...

Density of Langerhans cells in the keratocystic odontogenic tumor / Densidade das células de Langerhans no tumor odontogênico queratocístico

INTRODUCTION: Keratocystic odontogenic tumors (KOTs) are distinct odontogenic lesions commonly affecting the mandible bones. Langerhans cells (LCs) are specialized dendritic cells responsible for the presentation of antigens to T lymphocytes in mucosal and cutaneous surfaces. OBJECTIVE: This study analyzed the immunohistochemical expression of LCs in KOTs. MATERIALS AND METHODS: Fifteen cases of KOTs were studied using the anti-CD1a marker. Results: LCs were observed in all 15 cases analyzed. They were found to be concentrated in areas of cystic epithelial hyperplasia, mainly in those areas presenting higher concentration of inflammatory cells. Furthermore, a significant association between the number of LCs and areas of cystic epithelium presenting hyperplasia (Mann-Whitney test, p = 0.0223) was observed. The shape and location of these cells in KOTs epithelium were variable. CONCLUSION: The lower number of LCs observed on atrophic cystic epithelium of KOTs may be due to decreased epithelial immunosurveillance and this may result in locally aggressive invasiveness.

INTRODUÇÃO: Tumor odontogênico queratocístico (TOQ) é uma lesão odontogênica de caráter distinto que afeta frequentemente ossos maxilares. Células de Langerhans (CLs) são células dendríticas especializadas, responsáveis pela apresentação de antígenos aos linfócitos T nas superfícies cutânea e mucosa. OBJETIVO: Este estudo analisou a expressão imuno-histoquímica das CLs em lesões de TOQ. MATERIAIS E MÉTODOS: Quinze casos de TOQ foram estudados utilizando o marcador anti-CD1a. RESULTADOS: As CLs foram observadas em todos os 15 casos analisados. Essas células estavam concentradas em áreas de hiperplasia do epitélio cístico, especialmente naquelas que apresentavam alta concentração de células inflamatórias. Em adição, foi encontrada associação significativa entre número de CLs e áreas do epitélio cístico que apresentavam hiperplasia (Mann-Whitney test, p = 0.0223). O formato e a localização dessas células no epitélio dos TOQs foram variáveis. CONCLUSÃO: O menor número de CLs encontrado no revestimento cístico atrófico dos TOQs pode ser atribuído à imunovigilância deficiente e isso pode resultar em comportamento biológico localmente agressivo.

Alteraciones maxilofaciales en histiocitosis de células de Langerhans: reporte de un caso / Maxillofacial abnormalities in Langerhans cell histiocytosis: a case report

La Histiocitosis de células de Langerhans conocida anteriormente como Histiocitosis X, es un término aplicado a un grupo de desórdenes poco comunes del sistema fagocítico mononuclear, caracterizado por una acumulación clonal y proliferación anormal de células de Langerhans derivadas de la medula ósea. El propósito de esta investigación es reportar las alteraciones maxilo-faciales en un paciente con diagnostico de Histiocitosis de Células de Langerhans. Se presenta el caso de un varón de tres años de edad con diagnostico de Histiocitosis de Células de Langerhans con afección ósea multifocal y otros sitios especiales, con antecedentes de otitis media supurativa recurrente, diabetes insípida, dermatitis seborreica y lesiones hipercrómicas en miembros inferiores. La exploración extraoral evidenció lesiones eritematosas y descamativas en piel de cara y cuero cabelludo; intraoralmente se observó movilidad en dientes posteriores de ambos maxilares acompañada de recesión gingival, caries dental y erupción prematura del 26. Radiograficamente presentó lesiones osteolíticas en alas mayores de esfenoides, techo orbitario izquierdo, pared orbitaria externa derecha, arco cigomático, peñasco del temporal y zonas posteriores de ambos maxilares, además de agenesia de los gérmenes dentarios del 35 y 45. Adicionalmente al tratamiento sistémico de quimioterapia se realizó control de la infección dentobacteriana y tratamiento quirúrgico de las zonas maxilares afectadas. Se concluye que los maxilares al igual que los huesos del cráneo deben ser evaluados en pacientes con diagnóstico de Histiocitocis de Células de Langerhans, ya que estos sitios pueden ser los únicos huesos afectados como sucedió en el caso estudiado

Langerhans Cell Histiocytosis, known earlier as Histiocytosis X is a term applied to a group of rare disorders of the reticuloendothelial system, characterized by an abnormal proliferation of Langerhans cells derived from bone marrow. The purpose of this research is to report the maxillofacial alterations of a patient with diagnostic of Langerhans Cell Histiocytosis. A three-year old male patient with diagnostic of Langerhans Cell Histiocytosis with multifocal bone involvement and special sites of disease is presented. He had a previous history of exophtalmus, recurrent suppurative otitis media, diabetes insipidus, seborrheic dermatitis and hyperchromic lesions in lower members. The extraoral exam showed erythematosus and desquamative lesions in face skin and the scalp, intraorally back teeth mobility of both maxilars was found together with gingival recession, dental caries and premature eruption of 26. Radiographically, he presented osteolitic lesions in the sphenoid major wings, left orbital roof, right external orbital wall, zygomatic arch, temporal bone and back areas of both maxilars, besides dental germ agenesia of 35 and 45. In addition to the systemic chemiotherapy treatment, a control of the dentobacterial infection and surgical treatment of the affected maxillary areas was made. The conclusion is that maxilars as well as the skull bones must be evaluated in patients with a diagnostic of Langerhans cell Histiocytocis, because this could be the only bone involvement to be found as it happened in this case

Interstitial and Langerhans' dendritic cells in chronic periodontitis and gingivitis

The aim of the present study was to compare quantitatively the distribution of dendritic cell subpopulations in chronic periodontitis and gingivitis. Fourteen biopsies from patients with chronic periodontitis and fifteen from patients with gingivitis were studied. An immunoperoxidase technique was used to quantify the number of Langerhans' cells (CD1a) and interstitial dendritic cells (factor XIIIa) in the oral and sulcular and junctional/pocket epithelia and in the lamina propria. A greater number of factor XIIIa+ dendritic cells in the lamina propria and CD1a+ dendritic cells in the oral epithelium were observed in gingivitis compared to the periodontitis group (p = 0.05). In the sulcular and junctional/pocket epithelia and in the lamina propria, the number of CD1a+ dendritic cells was similar in the gingivitis and periodontitis groups. In conclusion, the number of Langerhans' cells in the oral epithelium and interstitial dendritic cells in the lamina propria is increased in gingivitis compared to periodontitis, which may contribute to the different pattern of host response in these diseases.

Wegener's granulomatosis--an etiology of acute pancreatitis.

Wegener's granulomatosis is a systemic disease that usually involves the upper respiratory tract and kidneys. We report a 47-year-old man with Wegener's granulomatosis that presented as acute pancreatitis.

Langerhans cell histiocytosis of skin: a clinicopathologic analysis of five cases.

BACKGROUND AND AIMS: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. METHODS: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. RESULTS: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells) with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. CONCLUSION: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.

Primary oral tuberculosis: report of two cases.

Oral lesions of tuberculosis though uncommon, are seen in both the primary and secondary stages of the disease. In secondary tuberculosis, the oral manifestations may be accompanied by lesions in the lungs, lymph nodes, or in any other part of the body and can be detected by a systemic examination. Primary oral tuberculosis may present as a diagnostic challenge for the clinician. Here we report two patients with primary tuberculosis in the oral cavity who presented to the dental department, were diagnosed and referred for medical management.

Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.

Conociendo a la célula de Langerhans / Meet the Langerhans cell

La célula de Langerhans (CL) representa uno de los elementos más eficaes del sistema inmunológico. Presentes desde las primeras etapas de la vida embrionaria, no sólo endocitan, procesan a los antígenos tópicos o ambientales, sino que tienen la capacidad de migrar hacia ganglios linfáticos para presentar los mismos a los linfocitos TCD4. Si bien su estructura y funcionalidad aún no están bien establecidas, se conoce que el gránulo de Bierbeck posee entre otras una proteína denominada Langerina, la cual estaría implicada en el proceso de endocitosis. Muchas líneas de investigación están abocadas al análisis de estos elementos, debido a que podrían estar implicadas en la génesis de diferentes neoplasias como linfomas, melanomas y carcinoma de cuello uterino, y a partir de ésto, la generación de vacunas antitumorales es un área donde el desarrollo de la inmunología, aunada a la biología molecular, avanzan en forma continua