Abstract:Kindler syndrome is a rare autosomal recessive genodermatosis characterized by trauma-induced blisters, progressive poikiloderma and varying degrees of photosensitivity. In 2003, loss-of-function mutations were identified in the gene KIND1 mapped to chromosome 20p12.3. In this paper, we report Kindler syndrome in two children born to consanguineous parents presenting acral blistering, photosensitivity, poikiloderma, cutaneous atrophy and periodontitis. Keywords: Clinical diagnosis; Epidermolysis bullosa; Photosensitivity disorders; Signs and symptoms Resumo: A síndrome de Kindler é uma genodermatose rara, autossômica recessiva, caracterizada pela presença de bolhas induzidas por traumas, fotossensibilidade, atrofia cutânea e poiquilodermia progressiva. A alteração genética da síndrome foi descrita em 2003, com a identificação de mutação no gene KINDIN1, localizado no cromossomo 20p12.3. Nesse trabalho relata-se a presença da síndrome de Kindler em irmãos, filhos de pais consangüíneos, que apresentavam, desde a infância, fotossensibilidade, bolhas após pequenos traumas, poiquilodermia, atrofia cutânea e periodontite.

Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma.

Barraquer-Simons syndrome, also called acquired partial lipodystrophy or cephalothoracic lipodystrophy, is a rare form of progressive lipodystrophy, characterized by symmetrical lipoatrophy of subcutaneous adipose tissue starting in the head and spreading to the thorax, upper and lower limbs and thighs. In this work, we report the case of a patient with Barraquer-Simons syndrome without systemic complications.

Abstract:We report the case of a patient with cutaneous and arthropathic psoriasis for eight years, whose sudden increased severity of the clinical picture led to a diagnosis of AIDS, emphasizing the importance of the specialist in the diagnosis of systemic diseases. The appropriate therapeutic interventions, including antiretroviral therapy and the immunobiological agent etanercept, enabled rapid improvement of symptoms and quality of life of the patient, besides contributing to delay in immunodeficiency progression. Keywords: Acquired immunodeficiency syndrome; Arthritis psoriatic; Psoriasis; Therapeutics Resumo: Relata-se o caso de um paciente com psoríase cutânea e artropática há oito anos, cujo agravamento súbito do quadro clínico levou ao diagnóstico da Aids, ressaltando a importância do especialista no diagnóstico de doenças sistêmicas. A intervenção terapêutica apropriada, incluindo a terapia antirretroviral e agente imunobiológico -etanercepte -possibilitou a rápida melhora do quadro clínico e da qualidade de vida do paciente, além de contribuir para o retardo na progressão da imunodeficiência.