Proteus syndrome

Abstract: Proteus syndrome is a rare syndrome characterized by disproportionate overgrowth of limbs, multiple hamartomas, and vascular malformations. The cerebriform connective tissue nevi, also called cerebriform plantar hyperplasia, are present in most patients, and is the main characteristic of the syndrome. If present, even alone, they can be considered as a pathognomonic sign. This article reports a classic case of Proteus syndrome in a 2-year-old male patient who began to show a discrete asymmetry of the right hemibody in relation to the left one after birth, which increased over the months. He also showed cerebriform plantar hyperplasia and Port-wine stains, among other alterations.

Disseminated cryptococcosis manifested as a single tumor in an immunocompetent patient, similar to the cutaneous primary forms

Abstract Cryptococcosis is a fungal infection caused by Cryptococcus neoformans that tends to affect immunocompromised individuals. The fungi are mostly acquired by inhalation, which leads to an initial pulmonary infection. Later, other organs - such as the central nervous system and the skin - can be affected by hematogenous spread. In addition, cutaneous contamination can occur by primary inoculation after injuries (primary cutaneous cryptococcosis), whose diagnosis is defined based on the absence of systemic involvement. The clinical presentation of cutaneous forms typically vary according to the infection mode. We report an unusual case of disseminated cryptococcosis in an immunocompetent patient with cutaneous lesions similar to those caused by primary inoculation. This clinical picture leads us to question the definition of primary cutaneous cryptococcosis established in the literature.

Associação da forma atípica da síndrome de Cogan com PR3-ANCA. Relato de caso / An association with features of atypical Cogan?s syndrome and PR3-ANCA. Case report

JUSTIFICATIVA E OBJETIVOS: A síndrome de Cogan caracteriza-se pela presença de ceratite não luética associada à disfunção cócleo-vestibular. Pode ter várias outras manifestações clínicas, como: mialgia, febre e vasculite. Menos de 300 casos foram relatados até fevereiro do corrente ano e nenhum publicado na literatura mundial, apresentando positividade do marcador c-ANCA associado à antiproteinase PR3. O objetivo do presente estudo foi alertar os colegas médicos sobre essa entidade nosológica pouco conhecida, que exige um tratamento precoce imunossupressor, com a finalidade de melhorar o prognóstico. RELATO DO CASO: Paciente do sexo masculino, 43 anos, branco, casado, comerciário, natural de Piratini (RS) e procedente de sua residência, após ter ficado hospitalizado por 10 dias e recebido alta sem diagnóstico. Destaca-se descrição de síndrome clínica rara iniciada por comprometimento ocular (olho vermelho), mialgias, febre e, 30 dias após, quadro sugestivo de comprometimento do oitavo par craniano, de aparecimento súbito, caracterizado por nistagmo, náuseas, vômitos e ataxia para a marcha. Durante a internação, associou-se o aparecimento de sufusões subungueais dolorosas compatíveis com vasculite sistêmica. CONCLUSÃO: O estudo do diagnóstico diferencial sobre vasculites auxiliou decisivamente a conclusão diagnóstica final; inclusive a resposta terapêutica com pulsoterapia (metilprednisolona 1g, por via venosa durante quatro dias) foi eficaz. Os sinais e os sintomas desapareceram em 24h.