Abcesso cerebral num doente com Rendu-Osler-Weber / Cerebral abscess in a patient with Rendu-Osler-Weber

A telangiectasia hemorrágica hereditária é uma doença familiar rara, descrita pela primeira vez no final do século 19, caracterizada por telangiectasias mucocutâneas e viscerais, e por malformações arteriovenosas. Manifestações neurológicas ocorrem em 8 a 12% dos doentes e resultam em mais de metade dos casos de malformações arteriovenosas pulmonares. A telangiectasia hemorrágica hereditária é uma das causas mais frequentes de abcessos cerebrais recorrentes, múltiplos e bilaterais, tendendo estes a ocorrerem na 3a para a 5a década. Apresentamos o caso de uma mulher de 88 anos admitida por prostração, febre e insuficiência respiratória, com a suspeita de metastização pulmonar de neoplasia oculta, cuja investigação revelou, entretanto, um abcesso cerebral secundário a malformações arteriovenosas pulmonares. Apesar da antibioterapia e da drenagem cirúrgica do abcesso, a paciente veio a falecer. O prognóstico após drenagem dos abcessos sem ressecção das lesões pulmonares é desfavorável, podendo sua elevada mortalidade refletir um atraso diagnóstico pela falta de reconhecimento dessa associação.

Hereditary hemorrhagic telangiectasia is a rare familial disorder, first described in the late 19th century characterized by multiple mucocutaneous and visceral telangiectases and arteriovenous malformations. Neurologic manifestations occur in 8 to 12% of patients and result from pulmonary arteriovenous malformations in 50% of cases. Hereditary hemorrhagic telangiectasia is one of the most frequent causes of recurrent, multiple and bilateral cerebral abscess and these tend to occur in the 3rd to 5th decade. We present the case of an 88-year-old woman admitted for prostration, fever and respiratory failure who was thought to have pulmonary metastasis of an unknown tumor but investigation revealed a brain abscess secondary to pulmonary arteriovenous malformations. Despite antibiotics and surgical drainage the patient died. The prognosis of brain abscess drainage without resection of the pulmonary lesions is not favorable. The high mortality of brain abscess in these patients may reflect a delay in diagnosis due to lack of recognition of this association.

Neutrophilic dermatosis of the dorsal hands associated with chronic hepatitis C virus infection.

Neutrophilic dermatosis of the dorsal hands, a localized form of Sweet's syndrome, was recently described, and can be associated with several diseases including infections. Chronic hepatitis C virus infection has been proposed as a possible triggering factor. The authors present a case in which the clinical and laboratory workup diagnosis only revealed positive serology for hepatitis C virus. Although a cause-effect relation could not be proved, it might be advisable to include serology for this virus in the initial evaluation of patients with neutrophilic dermatosis.