Deborah Aben-Athar Unger

Líquen escleroso e atrófico acral: relato de caso

O liquen escleroso e atrofico e uma doenca cronica da pele e mucosas, que, usualmente, afeta a area genital de mulheres. Poucos casos de liquen escleroso e atrofico, confinados nos pes e/ou maos, tem sido relatados. Relatamos um caso de liquen escleroso e atrofico envolvendo as extremidades e,posteriormente, a area perigenital

Plasmacytoid dendritic cells in cutaneous lesions of patients with chromoblastomycosis, lacaziosis, and paracoccidioidomycosis: a comparative analysis

Plasmacytoid dendritic cells (pDCs) are characterized by expression of CD123 and BDCA-2 (Blood Dendritic Cell Antigen 2) (CD303) molecules, which are important in innate and adaptive immunity. Chromoblastomycosis (CBM), lacaziosis or Jorge Lobos disease (JLD), and paracoccidioidomycosis (PCM), are noteworthy in Latin America due to the large number of reported cases. The severity of lesions is mainly determined by the hosts immune status and in situ responses. The dendritic cells studied in these fungal diseases are of myeloid origin, such as Langerhans cells and dermal dendrocytes; to our knowledge, there are no data for pDCs. Forty-three biopsies from patients with CBM, 42 from those with JLD and 46 diagnosed with PCM, were evaluated by immunohistochemistry. Plasmacytoid cells immunostained with anti-CD123 and anti-CD303 were detected in 16 cases of CBM; in those stained with anti-CD123, 24 specimens were obtained from PCM. We did not detect the presence of pDCs in any specimen using either antibody in JLD. We believe that, albeit a secondary immune response in PCM and CBM, pDCs could act as a secondary source of important cytokines. The BDCA-2 (CD303) is a c-type lectin receptor involved in cell adhesion, capture, and processing of antigens. Through the expression of the c-lectin receptor, there could be an interaction with fungi, similar to other receptors of this type, namely, CD207 in PCM and CD205 and CD209 in other fungal infections. In JLD, the absence of expression of CD123 and CD303 seems to indicate that pDCs are not involved in the immune response.

Epidermólise bolhosa distrófica pruriginosa: relato de caso

A epidermolise bolhosa distrofica pruriginosa e doenca genetica rara cujo padrao de heranca ainda nao esta bem estabelecido na literatura. O defeito genetico, que envolve a codificacao do colageno tipo VII, esta localizado no braco curto do cromossomo 3, ocorrendo mutacao no gene COL7A1. Apresenta-se o caso de um paciente do sexo masculino que referia prurido nas pernas ha cerca de 15 anos, cujo diagnostico foi firmado com base nos exames dermatologico e imuno-histopatologico. Devido a raridade dessa condicao patologica, realiza-se breve revisao do tema.

Th17 and regulatory T cells contribute to the in situ immune response in skin lesions of Jorge Lobo's disease

Jorge Lobos disease (JLD) is a chronic granulomatous mycosis described in various Latin American countries. The main objective of the present study was to investigate the possible role of Th17 and Foxp3+ Treg cells in the pathogenesis of Jorge Lobos disease. Human skin biopsies were submitted to an immunohistochemistry protocol to detect Foxp3, interleukin (IL)-1beta, CD25, IL-6, IL-17, and IL-23. The epidermis presented acanthosis, hyperkeratosis, and frequent presence of fungi. The dermis presented inflammatory infiltrate comprising macrophages, lymphocytes, epithelioid and multinucleated cells, and an intense number of fungi. Foxp3+ Treg cells and IL-17+ cells were visualized in lymphocytes in the inflammatory infiltrate. IL-1, IL-2R (CD25), IL-6, and IL-23 were visualized in the dermis, intermingled with fungal cells, permeating or participating of the granuloma. Following IL-17, the most prominent cytokine was IL-6. IL-23 and cells expressing CD25 were present in fewer number. The comparative analysis between IL-17 and Foxp3 demonstrated a statistically significant increased number of IL-17+ cells. Th17 cells play a role in the immune response of JLD. IL-1beta and IL-6 added to the previously described increased number of TGF-beta would stimulate such pattern of response. Th17 cells could be present as an effort to modulate the local immune response; however, high levels of a Th17 profile could overcome the role of Treg cells. The unbalance between Treg/Th17 cells seems to corroborate with the less effective immune response against the fungus.

Hemangiomas extensos da infância tratados com propranolol: relato de dois caso

Hemangiomas sao os tumores benignos mais frequentes da infância, apresentando como historia natural crescimento rapido, seguido de uma fase de regressao que culmina com o desaparecimento parcial ou total da lesao. Opcoes terapeuticas devem ser avaliadas para casos extensos. Os glicocorticoides sistemicos sao a terapia de escolha; contudo, ha relatos de que o propranolol oferece resultados melhores e mais rapidos. Este trabalho descreve dois casos de hemangioma infantil de grande volume associados a limitacao funcional e desfiguracao estetica com significativa resposta ao propranolol, droga esta que surge como uma proposta terapeutica oferecendo resultados satisfatorios e mantidos, com poucos efeitos colaterais.

The cytotoxic T cells may contribute to the in situ immune response in Jorge Lobo's Disease human lesions.

Abstract Jorge Lobos Disease (JLD) is a cutaneous chronic granulomatous disease caused by the pathogenic fungus Lacazia loboi. It is characterized by a granulomatous reaction with multinucleated giant cells and high number of fungal cells. In order to contribute to the comprehension of immune mechanisms in JLD human lesions, we studied the cytotoxic immune response, focusing on TCD8+ and NK cells, and granzyme B. Forty skin biopsies of lower limbs were selected and an immunohistochemistry protocol was developed to detect CD8+ T cells, NK cells and Granzyme B. In order to compare the cellular populations, we also performed a protocol to visualize TCD4+ cells. Immunolabeled cells were quantified in nine randomized fields in the dermis. Lesions were characterized by inflammatory infiltrate of macrophages, lymphocytes, epithelioid and multinucleated giant cells with intense number of fungal forms. There was a prevalence of CD8 over CD4 cells, followed by NK cells. Our results suggest that in JLD the cytotoxic immune response could represent another important mechanism to control Lacazia loboi infection. We may suggest that, although CD4+ T cells are essential for host defense in JLD, CD8+ T cells could play a role in the elimination of the fungus.