Langerhans' cell histiocytosis diagnosed due to dermatological perianal lesion / Histiocitose de células de Langerhans diagnosticada por lesão perianal dermatológica

Abstract Langerhans' cell histiocytosis is a rare disease characterized by proliferation of Langerhans cells in the body. It affects mainly males, predominantly in childhood. Ulcerated plaques are one of the cutaneous forms of presentation. Diagnostic confirmation is done through immunohistochemistry. As therapeutic options, topical corticosteroids and chemotherapy are good choices. The case is reported of a male patient, aged 14, with perianal ulceration. He consulted a coloproctologist, who performed a biopsy of the region and started local triamcinolone applications. Immunohistochemistry diagnosed Langerhans' cells histiocytosis. Further investigation revealed diabetes insipidus, osteolytic lesions in the skull and lower limbs, enlarged liver, and encephalic alterations. Chemotherapy was started with Vinblastine, with significant improvement of the lesions.

Resumo A histiocitose de células de Langerhans é uma doença rara caracterizada pela proliferação de células de Langerhans no corpo. A doença afeta principalmente os homens, predominantemente na infância. Placas ulceradas são uma das formas cutâneas de apresentação. A confirmação diagnóstica é feita através de análise imuno-histoquímica. Como opções terapêuticas, corticosteroides tópicos e quimioterapia são boas escolhas. O caso aqui relatado é de um paciente do sexo masculino, com idade de 14 anos, com ulceração perianal. Ele consultou um coloproctologista, que realizou uma biópsia da região e iniciou o tratamento com aplicações locais de triancinolona. A análise imunohistoquímica diagnosticou histiocitose de células de Langerhans. Outros exames revelaram diabetes insipidus, lesões osteolíticas no crânio e nos membros inferiores, aumento do fígado e alterações encefálicas. A quimioterapia foi iniciada com vimblastina, com melhora significativa das lesões.

Retículo-histiocitose congênita autolimitada em recém-nascido (Hashimoto- Pritzker) / Congenital self healing reticulohistiocytosis in a newborn (Hashimoto Pritzker)

A retículo-histiocitose congênita autolimitada é o espectro benigno das histiocitoses de células de Langerhans, caracterizada pela presença de lesões cutâneas ao nascimento ou no período neonatal, ausência de manifestações sistêmicas e resolução espontânea do quadro clínico. Apesar do curso benigno e frequente autorresolução na maior parte dos pacientes, estudos mostram que, em alguns casos, pode haver disseminação ou recaída da doença, enfatizando que o curso clínico é variável, havendo necessidade de seguimento em longo prazo. O acompanhamento do paciente por longo período é importante para detectar possível envolvimento sistêmico, pois existe relato de recorrência, envolvendo pele, mucosa, ossos e glândula pituitária.

Congenital self-healing reticulohistiocytosis is the benign spectrum of Langerhans Cell Histiocytosis, characterized by cutaneous lesions at birth or in the neonatal period, absence of systemic manifestations and spontaneous resolution of clinical status. Despite the benign and often self-resolving course in most patients, studies show that in some cases there may be metastasis or recurrence of the disease, emphasizing that the clinical course is variable, requiring long-term follow-up. The monitoring of the patient for a long period is important to detect possible systemic involvement, as there is a report of recurrence involving the skin, mucosa, bone and pituitary gland.

E-cadherin and CD1a expression in gingival epithelium in periodontal health, disease and post-treatment.

Background: Epithelial integrity is important for maintenance of periodontal health. It is not fully known if non-surgical periodontal therapy is capable of recreating the epithelial barrier in its functional state. Patients and Methods: Sixty-five patients (31 males and 34 females) were included in the study. They were divided into group A (healthy gingiva 16 patients), group B (gingivitis 17 patients), group C (periodontitis 17 patients), and group D (post-treatment 15 patients). Gingival samples were collected and immunohistochemical study was done using E-cadherin and CD1a antibody. Statistical analysis was done using analysis of variance (ANOVA), followed by Tukey-Kramer multiple comparison test for CD1a and Tukey's highly significant difference (HSD) test for E-cadherin. Result: There was a statistically significant difference (P<0.001) in the expression of E-cadherin between healthy (1.846±0.555), gingivitis (1.100±0.994), and periodontitis group (0.700±0.483). Similarly, there was a statistically significant difference (P<0.001) in the expression of CD1a between healthy (75.70±3.09), gingivitis (42.53±3.09), and periodontitis group (29.07±3.08). However, the expression of E-cadherin (1.242±0.653) and CD1a in post-treatment samples (52.18±2.90) was lower with no statistically significant difference when compared to health. Discussion: The significant reduction in E-cadherin and CD1a levels in periodontal disease when compared to health could possibly be a result of invasion by the periodontopathogens and its subsequent sequel. Although, the post-treatment samples showed significant improvement when compared to disease, the reduction in E-cadherin and CD1a levels when compared to gingival health suggests that the epithelial barrier was not yet fully established in its functional state.

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.

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.

Relationship between Dendritic Cells and Activated Eosinophils in Induced Sputum of Asthmatics

It has been suggested that dendritic cells (DCs) are critical antigen presenting cells for eosinophilic airway inflammation in a mouse model of asthma, and cysteinyl leukotrienes may play a role in DC trafficking in asthmatics. We investigated whether the number of DCs is increased in the induced sputum of both atopic and nonatopic asthmatics and is related to activated eosinophil count in the sputum. Sputum was induced by inhalation of hypertonic saline in 9 atopic and 12 nonatopic asthmatics and 10 nonatopic normal controls, and differential cell counts were performed. DCs and activated eosinophils were identified by immunocytochemistry with monoclonal antibodies (anti-CD1a and EG2, respectively). There were significantly higher percentages of eosinophils, EG2+ cells, and CD1a+ DC in the sputum of atopic and nonatopic asthmatics compared with normal controls, respectively. In asthmatics, the percentage of CD1a+ DC was significantly correlated with that of EG2+ cells (Rs=0.62, p=0.004). We demonstrated that the increased number of DCs was evident in the induced sputum of both atopic and nonatopic asthmatics, and the DC number was related to the activated eosinophil count, which suggests that DCs may contribute to the ongoing eosinophilic inflammation in asthmatic airways, and vice versa.