Challenging of treating patients with exfolliative cheilittis: Report of two cases

Cheilitis is a term given to the inflammation that occurs in the vermillion of the lips. The exfoliative type is an uncommon form of cheilitis, which is characterized by inflammation and desquamation of the lip. It can cause aesthetic problems and compromise daily eating and phonation. The aim of this paper is to describe two cases of exfoliative cheilitis in young persons under periods of emotional stress and parafunctional habits. A 22-year-old white male and an 18-year-old black female presenting edema, intense dryness, and slight desquamation on the vermilion of the lips. In the second case, fissures with bleeding were also observed. Oral lesions were associated with intense emotional stress. The diagnosis of both was made based on the clinical presentation and the exclusion of other conditions. Although the patients have presented a significant improvement after the corticosteroid treatment, they still have a recurrence in stressful episodes. Detailed clinical examination and complementary exams are fundamental for determining associated factors and correctly diagnosing exfoliative cheilitis. Treatment can be challenging, especially in the face of relapses. Key words:Cheilitis, exfoliative cheilitis, oral lesions, stress psychological

Validação da técnica das molduras adesivas para a quantificação dos neurofibromas discretos e estudo da influência dos hormônios progesterona e 17β–estradiol no número e tamanho destes tumores provenientes de indivíduos com neurofibromatose tipo 1

neurofibromas cutâneos e subcutâneos, também denominados discretos, uma das suas principais manifestações clínicas. Apesar do número e tamanho dos neurofibromas serem bastante variáveis na NF1, uma técnica objetiva para quantificar estes múltiplos tumores inexiste. É sabido que os neurofibromas discretos surgem na puberdade e alteram-se na gravidez. Assim, a progesterona e o estrogênio têm sido os hormônios mais investigados. A maioria dos neurofibromas expressa o receptor da progesterona (RP), porém a minoria expressa o receptor do estrogênio (RE). A presença de polimorfismos em genes de receptores hormonais aumenta a expressão destas proteínas em algumas neoplasias, sendo que esta relação nunca foi investigada na NF1. Ademais, estudos envolvendo os níveis séricos hormonais são escassos. Objetivos Gerais: Validar a técnica das molduras adesivas para estimar o número de neurofibromas discretos. Investigar a relação dos hormônios progesterona e 17β-estradiol com o número e tamanho destes neurofibromas associados à Neurofibromatose tipo 1. Material e Métodos: O presente trabalho foi dividido em duas etapas, ambas compostas por 92 indivíduos com NF1. ETAPA 1: Os neurofibromas discretos foram quantificados pela técnica das molduras adesivas em 92 indivíduos e, em 49 indivíduos, foi realizada a contagem exata “um a um” destes tumores com o intuito de avaliar a capacidade preditiva da técnica. O coeficiente de correlação intraclasse (CCI), teste t pareado e gráficos de Bland-Altman e concordância-sobrevivência foram aplicados na análise interexaminador (dois avaliadores) da técnica, sendo a correlação de Spearman e regressão linear usados para avaliar a representatividade da técnica das molduras adesivas. ETAPA 2: Cada indivíduo foi submetido à coleta de sangue e biópsia de dois neurofibromas discretos: o maior e o menor. A avaliação da expressão dos anticorpos RP, RE e Ki-67 (índice de proliferação celular) foi realizada pela técnica da imuno-histoquímica em cortes obtidos do tissue microarray (TMA) e tissue macroarray (TMaA). A imunoquantificação foi feita através do sistema computadorizado da Aperio (Leica Byosistem, EUA). O polimorfismo genético foi identificado pela reação em cadeia da polimerase (PCR). Os níveis séricos hormonais foram avaliados pelo ensaio de imunoabsorbância ligado à enzima. A significância estatística aceita foi de p ≤ 0,05. Resultados: Considerando a ETAPA 1, houve excelente concordância entre os avaliadores (CCI = 0.995) e o número total de neurofibromas discretos pode ser predito pela técnica das molduras adesivas através da fórmula: 38,6 + 10,3x. Na ETAPA 2, os níveis séricos do hormônio progesterona foram associados positivamente com o número de neurofibromas discretos. A expressão do RP foi identificada em 100% dos neurofibromas. O RE foi positivo em apenas 4% dos tumores. A expressão do RP foi associada ao índice de proliferação celular, estando presente de forma significativa nos neurofibromas subcutâneos. Conclusão: A técnica das molduras adesivas prediz adequadamente o número de neurofibromas discretos na NF1. A progesterona tanto apresenta associação positiva com o número de neurofibromas, como seu receptor é correlacionado com o maior índice de proliferação celular e tipo clínico de tumor, demonstrando sua influência (hormônio e receptor) nos neurofibromasIntroduction: Neurofibromatosis type 1 (NF1) is a complex disease with cutaneous and subcutaneous neurofibromas, also called discrete, as one of its main clinical manifestations. Despite being quite variable in number and size among individuals with NF1, an objective technique to quantify the number of these multiple tumors does not exist. It is known that discrete neurofibromas arise in puberty and change in pregnancy. Thus, progesterone and estrogen hormones have most been investigated. While the majority of neurofibromas express progesterone receptor (PR), only few express the estrogen receptor (ER). The presence of polymorphisms in hormone receptors genes increases the expression of these proteins in some tumors, and this relation has never been investigated in NF1. Moreover, studies involving hormone serum levels are scarce. General aims: To validate the technique of paper frames to estimate the number of discrete neurofibromas. To investigate the relationship between the progesterone and 17β-estradiol hormones and the number and size of these neurofibromas associated with neurofibromatosis type 1. Material and Methods: The present study was divided into two phases, both compounds by 92 individuals with NF1. PHASE 1: The discrete neurofibromas were quantified by the technique of paper frames in 92 subjects, and 49 subjects, the exact "one by one" count of the neurofibromas was performed to evaluate the technique predictive capacity. The intraclass correlation coefficient (ICC), paired t-test, Bland-Altman and reliability-survival charts were applied in inter-examiners technique (two examiners) agreement, being the Spearman correlation and linear regression used to evaluate the representativeness of the paper frames technique. PHASE 2: Each subject underwent blood sampling and biopsy of two discrete neurofibromas: the large and small. The evaluation of the PR, ER and Ki-67 (cell proliferation) antibodies expression was performed by immunohistochemistry in obtained sections of tissue microarray (TMA) and tissue macroarray (TMaA). Immunohistochemical quantification was performed using Aperio computerized system (Leica Byosistem, USA). Polymorphism was identified by polymerase chain reaction (PCR). Immunoabsorbance testing assessed serum hormonal levels. Statistical significance is accepted with p-value ≤ 0.05. Results: Regarding Phase 1, there was excellent agreement between examiners (ICC = 0.995) and the total number of discrete neurofibromas can be predicted by the paper frames technique using the formula: 38.6 + 10.3x. In Phase 2, the serum hormonal levels of progesterone were positively associated with number of neurofibromas. The PR expression was observed in 100% of discrete neurofibromas. The ER was positive in only 4% of the tumors. The expression of PR was associated with cell proliferation and is significantly present in subcutaneous neurofibromas. Conclusion: The technique of paper frames adequately predicts the number of discrete neurofibromas in NF1. Progesterone is associated with number of neurofibromas, as well as its receptor played positive association in rate of cell proliferation and is significantly expressed in subcutaneous neurofibromas, showing their (hormone and receptor) influence on these tumors258f

Gingival Anaplastic Large-Cell Lymphoma Mimicking Hyperplastic Benignancy as the First Clinical Manifestation of AIDS: A Case Report and Review of the Literature

This paper presents an unusual case of gingival ALCL, which mimicked a benign hyperplastic lesion that occurred in a 57-year-old white man representing the first clinical manifestation of acquired immunodeficiency syndrome (AIDS). The patient was referred to the Dental Clinic of PUCPR complaining of a lobulated nodule on the gingiva of his upper central incisors. The presence of advanced chronic periodontitis and dental plaque raised suspicion for a benignancy. An excisional biopsy was performed, and large pleomorphic cells with an abundant cytoplasm, sometimes containing prominent nucleoli and “Hallmark” cells, were observed through hematoxylin and eosin staining. The tumor cells showed strong CD30 expression, EMA, Ki-67, and LCA, and negative stain for p80NPM/ALK, CKAE1/AE3, CD20, CD3, CD56, and CD15. The final diagnosis was ALCL (ALK-negative). Further laboratory tests revealed positivity for human immunodeficiency virus (HIV). The patient was submitted to chemotherapy, but four months after diagnosis, the patient died due to pneumonia and respiratory failure. Oral anaplastic large-cell lymphoma (ALCL) is a rare disorder. Only 5 cases involving the gingiva have been reported, and to our knowledge, this is the first case reported of the ALCL, which mimicked a hyperplastic benignancy as the first clinical manifestation of AIDS