Histiocitosis de células de Langerhans: seguimiento de 5 años del caso y revisión sistemática de literatura / Histiocitose de células de Langerhans: acompanhamento de 5 anos e revisão sistemática da literatura / Langerhans cell histiocytosis: 5-year follow-up and systematic literature review

La Histiocitosis de células de Langerhans (HCL), es una enfermedad rara, caracterizada por la proliferación clonal agresiva de células de Langerhans dendríticas de la médula ósea. La enfermedad crea lesiones osteolíticas en donde las manifestaciones orales usualmente están presentes. El objetivo de este reporte de caso es, presentar el seguimiento de cinco años de un caso diagnosticado en el 2014 y una revisión sistemática de literatura de las manifestaciones orales de HCL. Para la revisión sistemática, el argumento de búsqueda con palabras claves como HCL, histiocitosis X y manifestaciones orales, fueron conducidas en cinco bases de datos como PUBMED, LILACS, EBSCO, OVID y SCOPUS. Las manifestaciones orales más comunes son inflamación, enrojecimiento y pérdida de hueso en las áreas afectadas. Es importante mantener un seguimiento periódico en el consultorio dental de los casos de HCL en la población pediátrica. La HCL usualmente presenta manifestaciones orales que pueden ser un signo de diagnóstico temprano de esta condición, especialmente en la población pediátrica.

A histiocitose de células de Langerhans (HCL) é uma doença rara caracterizada pela proliferação clonal agressiva de células dendríticas de Langerhans da medula óssea. A doença cria lesões osteolíticas onde as manifestações orais geralmente estão presentes. O objetivo deste relato de caso é apresentar o seguimento de cinco anos de um caso diagnosticado em 2014 e uma revisão sistemática da literatura sobre as manifestações orais da HCL. Para a revisão sistemática, a busca de argumento com palavras-chave como LCH, histiocytosis X e oral manifestations, foi realizada em cinco bases de dados como PUBMED, LILACS, EBSCO, OVID e SCOPUS. As manifestações orais mais comuns são inflamação, vermelhidão e perda óssea nas áreas afetadas. É importante monitorar regularmente os casos de HCL na população pediátrica no consultório odontológico. A HCL geralmente apresenta manifestações orais que podem ser um sinal diagnóstico precoce dessa condição, principalmente na população pediátrica

Langerhans cell histiocytosis (LCH) is a rare disease characterized by aggressive clonal proliferation of dendritic Langerhans cells from the bone marrow. The disease creates osteolytic lesions where oral manifestations are usually present. The objective of this case report is to present the five-year follow-up of a case diagnosed in 2014 and a systematic review of the literature on the oral manifestations of LCH. For the systematic review, the search argument with keywords such as LCH, histiocytosis X and oral manifestations, were conducted in five databases such as PUBMED, LILACS, EBSCO, OVID and SCOPUS. The most common oral manifestations are inflammation, redness, and bone loss in the affected areas. It is important to regularly monitor LCH cases in the pediatric population in the dental office. LCH usually presents oral manifestations that can be an early diagnostic sign of this condition, especially in the pediatric population

Immunohistochemical analysis of S100-positive epidermal Langerhans cells in dermatofibrom

Abstract Dermatofibroma is a dermal fibrohistiocytic neoplasm. The Langerhans cells are the immunocompetent cells of the epidermis, and they represent the first defense barrier of the immune system towards the environment. The objective was to immunohistologically compare the densities of S100-positive Langerhans cells in the healthy peritumoral epidermis against those in the epidermis overlying dermatofibroma (20 cases), using antibodies against the S100 molecule (the immunophenotypic hallmark of Langerhans cells). The control group (normal, healthy skin) included ten healthy age and sex-matched individuals who underwent skin biopsies for benign skin lesions. A significantly high density of Langerhans cells was observed both in the epidermis of the healthy skin (6.00 ± 0.29) and the peritumoral epidermis (6.44 ± 0.41) vs. those in the epidermis overlying the tumor (1.44 ± 0.33, p < 0.05). The quantitative deficit of Langerhans cells in the epidermis overlying dermatofibroma may be a possible factor in its development.

Histiocitosis congénita de células de Langerhans / Congenital Langerhans cell histiocytosis

La histiocitosis de células de Langerhans es una enfermedad poco frecuente, cuyas manifestaciones clínicas pueden aparecer en el periodo neonatal y varían desde lesiones óseas aisladas hasta un compromiso sistémico. Se describe un caso de histiocitosis de células de Langerhans y se revisa la literatura médica sobre las manifestaciones clínicas, el diagnóstico y el tratamiento. El paciente de un mes de nacido fue llevado a consulta por presentar adenopatías y lesiones en la piel que, inicialmente, fueron tratadas como reacción a una infección. La enfermedad continuó su progresión sin que hubiera mejoría con el tratamiento, hasta que el paciente falleció por falla respiratoria. La biopsia de ganglio linfático y la de piel revelaron infiltración de células atípicas, y la inmunohistoquímica resultó positiva para las proteínas S1OO, CD1 y CD68, con lo cual se confirmó el diagnóstico de histiocitosis de células de Langerhans. Esta alteración representa un gran desafío clínico, por lo que es importante alertar y sensibilizar al equipo médico para lograr un diagnóstico y un tratamiento más oportunos.

Langerhans cell histiocytosis is a rare pathology with different clinical manifestations in the neonatal period ranging from isolated bone lesions to systemic compromise. We report a case of Langerhans cell histiocytosis including a literature review focused on the clinical manifestations, diagnosis, and treatment. A one-month-old patient was brought to medical consultation with lymphadenopathy and skin lesions, which were initially managed as an infectious pathology. The disease continued its progression without improvement with the treatment until the patient died due to respiratory failure. The lymph node and skin biopsies revealed infiltration of atypical cells with positive immunohistochemistry for S1OO, CD1, and CD68 confirming Langerhans cell histiocytosis. This disorder represents a great challenge and, therefore, it is important to alert and sensitize medical teams about it for timely diagnosis and management.

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.

Presence of langerhans cells, regulatory T cells (Treg) and mast cells in asymptomatic apical periodontitis

Abstract Asymptomatic Apical Periodontitis is essentially an inflammatory disease of microbial aetiology. Association and function of the cell components involved, or specific inductive factors and growth mediators associated with development, maintenance and resolution of the periapical lesions are still unknown. The objective of this study was to evaluate the concentration of Regulatory T cells (FoxP3+; Treg), Langerhans cells (CD1a+; LC) and mast cells in asymptomatic apical periodontitis. 73 cases were selected: 30 periapical granulomas, 29 radicular cysts and 14 residual cysts. All groups were submitted to morphological analysis for classification of inflammatory infiltrate and thickness of the epithelial lining as well as to immunohistochemical analysis for detection of LC and Treg cells. Toluidine blue staining was used for detecting mast cells. Analysis showed higher mean numbers of LC (8.2 cells/0.2mm2), and Treg cells in radicular cysts (5.910 cells/0.2mm2). As for mast cells, it was found that radicular cysts had a higher mean number of these cells compared to other periapical lesions (12.68 cells/0.2mm2). The association between thickness of the epithelial lining and inflammatory cells showed that the presence of hypertrophic epithelium in radicular cysts presented higher density of LC. The number of LC and Treg cells play an important role in the control of the inflammatory micro-environment in periapical granulomas and radicular cysts, respectively. The presence of mast cells in radicular cysts may be associated with progression of the lesion. Knowledge regarding the inflammatory cell profile is therefore essential for a better understanding of the pathogenesis of asymptomatic periapical periodontitis.

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.

Expresión de E-cadherina y células de Langerhans en verruga vulgar y papiloma bucal / Expression of E-cadherin and Langerhans cells in verruca vulgaris and oral papilloma

Introducción: la verruga vulgar y el papiloma bucal son lesiones provocadas por el virus papiloma humano se pueden encontrar presentes en la mayoría de las superfi cies cutáneas y mucosas, su comportamiento biológico es benigno. Las proteínas de adhesión como la E-cadherina, se encargan de mantener la organización y morfología celular, disminuyen su expresión epitelial en ciertas lesiones potencialmente cancerizables, y favorecen la migración e invasión celular ocasionando posiblesmetástasis. Las células de Langerhans, son células presentadoras deantígeno que activan el sistema inmunológico para proteger al organismocontra patógenos o sustancias extrañas. Objetivo: Determinar el grado de expresión de E-cadherina y de células de Langerhans en verruga vulgar y papiloma bucal. Material y métodos: Se realizó un estudio descriptivo y retrospectivo, en el cual se incluyeron 16 bloques con tejido incluido en parafi na con diagnóstico de verruga vulgar, ycuatro diagnosticados como papiloma bucal, todos los casos fueron corroborados con la tinción de hematoxilina y eosina. La determinacióndel grado de expresión de las células de Langerhans y E-cadherina se realizó por medio de la técnica de inmunohistoquímica, la valoraciónse llevó a cabo de manera semicuantitativa, y se realizó estadísticadescriptiva. Resultados: La expresión de E-cadherina en verruga vulgar fue intensa tanto en cantidad como intensidad, mientras que en papiloma bucal fue moderada también en ambos casos; las células de Langerhans mostraron una inmunotinción moderada en las lesiones de verruga vulgar en intensidad y cantidad; en el caso de papiloma bucal esta inmunotinción en intensidad y cantidad fue leve. . Conclusiones:Los resultados mostraron que las proteínas de adhesión E-cadherinano pierden su expresión en la verruga vulgar y papiloma bucal, lo cualconfi rma su benignidad...

Introduction: verruca vulgaris and oral papilloma are lesions causedby the human papillomavirus. They can be found on most skin and mucosalsurfaces, and their biological behavior is benign. Adhesion proteinssuch as E-cadherins are responsible for maintaining cell morphologyand organization; they decrease the expression in certain potentiallycancerous epithelial lesions and promote cell migration and invasion,causing possible metastasis. Langerhans cells are antigen-presentingcells that activate the immune system to protect the body againstpathogens or foreign substances. Objective: To determine the degreeof expression of E-cadherin and Langerhans cells in verruca vulgarisand oral papilloma. Material and methods: We performed a descriptiveand retrospective study involving 16 paraffi n-embedded tissue blocksof diagnosed cases of verruca vulgaris and 4 paraffi n-embedded tissueblocks identifi ed as oral papilloma. The diagnosis was previouslyconfi rmed by hematoxylin and eosin staining. The degree of expressionof Langerhans cells and E-Cadherin was determined by immunohistochemistry,while the evaluation was carried out semiquantitatively,with descriptive statistics being performed. Results: The expression ofE-cadherin in verruca vulgaris was strong in terms of both quantityand intensity, whereas in the case of oral papilloma it was moderatefor both. Langerhans cells showed moderate immunostaining for bothintensity and quantity in verruca vulgaris-type lesions, while for oralpapilloma, the immunostaining was also mild in both cases. Conclusions:The results suggest that the expression of E-cadherin adhesionproteins does not diminish in verruca vulgaris and oral papilloma,which confi rms their benignity...

Corneal confocal microscopy in a healthy Brazilian sample / Microscopia confocal em córneas de brasileiros saudáveis

ABSTRACT Objective This study aims to evaluate the characteristics of the corneal sub-basal plexus by performing in vivo confocal microscopy of healthy Brazilians to provide reference values for the Brazilian population. Method This study is an observational, cross-sectional, descriptive study comparing corneas from 55 healthy Brazilian individuals across the age span of 20-70 years. Results The average number of fibers was 5.35 ± 1.36, fiber density was 33.4 ± 8.5 fibers per field, and the mean number of Langerhans cells was 5.13 ± 8.10. A correlation between the average number of fibers and age showed an inverse relationship between the number and density of fibers and age for women (p < 0.05). In the multivariate analysis, each annual increase of age showed an average increase of 1.017 (95%CI: 1.008 to 1.026) in the number of Langerhans cells, adjusting for sex and thickness. Conclusion Compared to other samples, this Brazilian population showed a higher average number of fibers, though further studies with a larger sample should be performed.

RESUMO Objetivo Este estudo tem como objetivo avaliar as características morfológicas do plexo sub-basal da córnea por microscopia confocal in vivo com indivíduos brasileiros saudáveis para fornecer uma referência para a população brasileira. Método Este trabalho é um estudo observacional, descritivo, transversal comparativo com microscopia confocal de córnea a partir de 55 indivíduos brasileiros saudáveis na faixa etária de 70 ± 20 anos de idade. Resultados Número médio de fibras foi 5,35 ± 1,36, a densidade foi de 33,4 ± 8,5 fibras por campo e de células de Langerhans foi 5,13 ± 8,10. Uma correlação entre o número médio de fibras e da idade dos indivíduos mostrou uma relação inversa entre o número e densidade de fibras, e idade para as mulheres, (p < 0,05). Na análise multivariada, cada aumento anual de idade apresentou um aumento médio de 1,017 (IC95%: 1,008-1,026) no número de células de Langerhans, ajustado para sexo e espessura. Conclusão Em comparação com outras amostras, esta população brasileira apresentou um maior número médio de fibras embora estudos com número maior de amostras necessitem ser realizados.

Langerhans Cell Histiocytosis of the petrous apex with inner ear involvement: Case report and literature review / Histiocitosis de células de langerhans del ápex petroso con compromiso del oído interno

Temporal bone involvement in Langerhans Cell Histiocytosis (LCH) is the second most common site of involvement in the head and neck area, with the mastoid and squamous portion of the bone as the most frequent site where LCH manifests. Since there are not many cases reported in the literature, it is possible to state that primary manifestation of histiocytosis affecting the inner ear structures is uncommon. This article reports the case of a patient with involvement of the petrous apex of the temporal bone that was referred to the Fundación Hospital de la Misericordia, and carries out a literature review in order to discuss the manifestation, treatment and outcome of this disease.

El compromiso del hueso temporal en la histiocitosis de células de Langerhans (HLC) es el segundo en frecuencia en el área de cabeza y cuello, siendo los sitios de mayor presentación la porción mastoidea y escamosa del hueso. La presentación primaria de la histiocitosis que afecta las estructuras del oído interno es poco frecuente, pues no hay muchos casos reportados en la literatura. En este artículo, se presenta un reporte de caso de compromiso del ápex petroso del temporal en una paciente remitida a la Fundación Hospital de La Misericordia y se realiza una revisión de la literatura para socializar la presentación de la enfermedad, su tratamiento y desenlace.

Enfermedad de Erdheim-Chester: fractura patológica de húmero izquierdo / Erdheim-Chester Disease: Pathological fracture of the left humerus

La Enfermedad de Erdheim-Chester es una forma rara de histiocitosis que no es de células de Langerhans. Es de etiología desconocida y compromete comúnmente a adultos. Las manifestaciones clínicas son heterogéneas debido a la infiltración histiocítica en múltiples sistemas. Cuando compromete al esqueleto produce dolor óseo, asociado a infiltrados pulmonares intersticiales, cardiacos, hepáticos o renales1-3. Describimos un caso que comienza con dolor óseo crónico y fractura patológica de húmero izquierdo con compromiso poliostótico, sin evidencia de enfermedad multisistémica.

The Erdheim Chester disease is a rare presentation of non Langerhans cell histiocytosis. The etiology is unknown, and is more common in adults. The clinical manifestations are heterogeneous due to histiocytic infiltration of multiple systems. When there is skeletal involvement, it produces bone pain, and may be associated with interstitial, heart, liver, kidney or pulmonary infiltrates1-3. A case is presented on a patient who began with chronic bone pain and a pathological fracture of the left humerus with polyostotic involvement and with no evidence of multisystemic disease.

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.

Histiocitosis de células de Langerhans en el adulto / Langerhans cell histiocytosis in adult

Las histiocitosis de células de Langerhans (HCL) son un grupo de enfermedades que presentan un comportamiento biológico variable y se caracterizan por la infiltración de uno o más órganos por células de Langerhans. Afecta principalmente a niños y es muy rara su presentación en la edad adulta. Puede comprometer piel, huesos, sistema nervioso, ganglios linfáticos, médula ósea, bazo e hígado. El tratamiento depende de la extensión y de la disfunción de los órganos comprometidos. Se presenta un paciente de 37 años con diagnóstico de HCL con compromiso cutáneo, óseo y de SNC...

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.

Immunohistochemical staining of Langerhans cells in HPV-positive and HPV-negative cases of oral squamous cells carcinoma

The Human Papillomavirus (HPV) has been strongly implicated in development of some cases of oral squamous cell carcinoma (OSCC). However, the immunological system somehow reacts against the presence of this virus. Among the cells involved in such mechanism of defense Langerhans cells (LC) stand out, which are responsible for processing and presenting antigens. OBJECTIVES: The purposes of this study were to investigate the presence of HPV DNA and to evaluate the immunohistochemical reactivity for Langerhans cells between HPV-positive and HPV-negative OSCC. Twenty-seven cases of OSSC were evaluated. MATERIAL AND METHODS: DNA was extracted from paraffin-embedded tissue samples and amplified by Polymerase Chain Reaction (PCR) for the detection of HPV DNA. Viral typing was performed by dot blot hybridization. Immunohistochemistry was performed by the Streptavidin-biotin technique. RESULTS: From the 27 cases, 9 (33.3 percent) were HPV-positive and 18 (66.0 percent) HPV-negative. HPV 18 was the most prevalent viral type (100 percent cases) and infection with HPV-16 (co-infection) was detected in only 1 case. In the OSCC specimens examined, immunoreactivity to S-100 antibody was detected in all cases, with a mean number of 49.48±30.89 Langerhans cells positive for immunostaining. The mean number of immunostained Langerhans cells was smaller in the HPV-positive cases (38 cells/case) than in the HPV-negative cases (42.5 cells/case), but this difference was not significant (p=0.38). CONCLUSIONS: The low frequency of detection of HPV DNA in OSCC indicates a possible participation of the virus in the development and progression of only a subgroup of these tumors. There was no association between the immunohistochemical labeling for Langerhans cells (S-100+) and HPV infection of in OSSC. These findings suggest that the presence of HPV in such OSCC cases could not alter the immunological system, particularly the Langerhans cells.

Imunopatologia da dermatite de contato alérgica / Immunopathology of allergic contact dermatitis

A dermatite de contato alérgica é consequência de uma reação imune mediada por células T contra químicos de baixo peso molecular, denominados haptenos. É uma condição frequente que ocorre em todas as raças e faixas etárias e afeta a qualidade de vida de seus portadores. O mecanismo imunológico desta doença vem sendo revisto nas últimas décadas com significativo avanço no seu entendimento. A metabolização e o caminho dos haptenos, bem como a formação e o mecanismo de ação das células responsáveis tanto pela reação quanto pelo seu término, são discutidos neste artigo.

Allergic contact dermatitis is the consequence of an immune reaction mediated by T cells against low molecular weight chemicals known as haptens. It is a common condition that occurs in all races and age groups and affects the quality of life of those who present it. The immunological mechanism of this disease has been reviewed in recent decades with significant advance in its understanding. The metabolism and pathway of the haptens as well as the activation and mechanism of action of the cells responsible for both the immune reaction and its completion are discussed in this article.