Hydroa Vacciniforme-Like T-Cell Lymphoma: A Further Brazilian Case.

Hydroa vacciniforme (HV)-like lymphoma is a rare, usually fatal Epstein-Barr virus-driven lymphoproliferative disease affecting children from Asia, Mexico, and South America. Cutaneous manifestations imitate HV, a benign photodermatosis in which systemic symptoms are not observed, and spontaneous regression occurs later in adolescence or young adulthood. We report a case of HV-like lymphoma in a 12-year-old girl, descendent from an ancient Amazon indigenous tribe that, as far as we know, represents the second Brazilian case ever reported in the medical literature.

Associação de vitiligo com autoanticorpos tireoidianos / Vitiligo and its association with thyroid autoantibodies

Objetivo: Analisar a presença de autoanticorpos antitireoidianos (anti-TPO) no soro de pessoas acometidas por vitiligo. Métodos: Estudo do tipo caso-controle retrospectivo realizado em serviço de dermatologia de referência na Amazônia, com amostra constituída por dois grupos: Grupo Vitiligo (n=56), com diagnóstico clínico de vitiligo, e Grupo Controle (n=30), que se declarou sadio, não portador de vitiligo, de outra dermatose e/ou doença autoimune diagnosticada. O registro dos dados foi feito pelo preenchimento de protocolo específico usado em entrevista para ambos os grupos, além de coleta de sangue para dosagem de autoanticorpos anti-TPO para os dois grupos. O teste qui quadrado e a odds ratio (OR) foram utilizados para variáveis qualitativas; para as quantitativas, foi utilizado o teste t de Student, e o nível de significância foi de p≤5%. Resultados: A história pessoal de doença autoimune esteve presente em 7,14% dos portadores versus 0% dos controles. Não houve diferenças estatisticamente relevantes com relação aos antecedentes familiares entre os grupos (OR: 0,5704; p=0,4146). Quanto à positividade para os autoanticorpos anti-TPO (níveis superiores ao ponto de corte), não houve relevância estatística (qui quadrado 2,844; p=0,229). Entretanto, na comparação dos níveis séricos absolutos de autoanticorpos anti-TPO entre os grupos, foram obtidos 129,49±323,88 para o portador da doença e 35,85±13,16 para o controle, com t=2,1602 e p=0,0351. Conclusão: Embora não tenha sido relevante a diferença entre os Grupo Vitiligo e Controle quanto à positividade para o autoanticorpos anti-TPO, ao se considerar a comparação com os valores séricos absolutos do Grupo Vitiligo, estes foram maiores que os apresentados pelos controles, sendo esta diferença estatisticamente relevante.(AU)

Objective: To analyze the presence of antithyroid autoantibodies (anti-TPO) in the serum of people affected by vitiligo. Methods: This is a study of retrospective casecontrol, performed in a reference dermatological center in Amazonia, with a sample consisting of two groups: Vitiligo group (n=56), with clinical diagnosis of vitiligo, and the Control Group (n=30), who was self-declared as healthy, nonvitiligo carrier, with no other dermatosis and/or diagnosed autoimmune disease. The data recording was made with specific protocol completion in an interview for both groups, and blood collection for antithyroid autoantibodies dosage for both groups. Chi-square and odds ratio (OR) tests were used for qualitative variables; for the quantitative ones, t-Student test, and significance level of p≤5%. Results: The personal history of autoimmune disease was present in 7.14% of patients compared to 0% of controls. There were no statistically significant differences in relation to family history between the groups (odds ratio: 0.5704; p=0.4146). As for the positivity for antithyroid autoantibodies (levels above the cutoff point), there was no statistical significance (chi-square=2.844, p=0.229). However, when comparing the absolute serum levels of antithyroid autoantibodies between the groups, 129.49±323.88 was obtained for the carrier of the disease, and 35.85±13:16 to controls, with t=2.1602, and p=0.0351. Conclusion: Although the difference between vitiligo and control groups were not significant regarding positivity for antithyroid autoantibodies, when the comparison with the absolute serum levels of the group with vitiligo was considered, they were higher than those presented by the controls, with this difference being statistically significant.(AU)

Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil.

BACKGROUND: Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. OBJECTIVES: To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients. METHODS: Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil. RESULTS: We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths. CONCLUSIONS: The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial.

Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil

BACKGROUND: Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. OBJECTIVES: To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients. METHODS: Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil. RESULTS: We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths. CONCLUSIONS: The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial. .

Sarcoid-like paracoccidioidomycosis presenting with perineural granuloma / Paracoccidioidomicose sarcoidica com granuloma perineural

Paracoccidioidomycosis presenting as a sarcoid-like plaque may be misdiagnosed as leprosy, especially when shared endemic areas are concerned. We report the case of a Brazilian male patient presenting with an ulcerated plaque on his left ear and neighboring areas. The plaque simulated tuberculoid leprosy type 1 reaction, both clinically and histopathologically. A perineural granuloma with no organisms detected by routine and Fite-Faraco staining reinforced that diagnosis. Paracoccidioidomycosis was confirmed only after a second biopsy, taken from the ulcerated area.

Paracoccidioidomicose expressando-se como placa sarcoídica pode ser confundida com hanseníase, especialmente em zonas endêmicas comuns às duas condições. Apresentamos um paciente brasileiro, com placa ulcerada na orelha direita e em áreas vizinhas que simulava, clinica e histopatologicamente, hanseníase tuberculoide em reação tipo 1. O encontro de granuloma perineural, sem parasitas detectáveis às colorações de rotina e Fite-Faraco, reforçou a hipótese de hanseníase. Apenas com uma nova biópsia, desta vez da área ulcerada, a paracoccidioidomicose pôde ser confirmada.

Sarcoid-like paracoccidioidomycosis presenting with perineural granuloma.

Paracoccidioidomycosis presenting as a sarcoid-like plaque may be misdiagnosed as leprosy, especially when shared endemic areas are concerned. We report the case of a Brazilian male patient presenting with an ulcerated plaque on his left ear and neighboring areas. The plaque simulated tuberculoid leprosy type 1 reaction, both clinically and histopathologically. A perineural granuloma with no organisms detected by routine and Fite-Faraco staining reinforced that diagnosis. Paracoccidioidomycosis was confirmed only after a second biopsy, taken from the ulcerated area.