Sarcoidosis is a multi-organ granulomatous disease of unknown etiology. Neurological involvement in sarcoidosis is uncommon but cranial mononeuropathies, especially involving the VII and VIII cranial nerves, are highly suggestive of neurosarcoidosis. We report the case of a 54-year-old woman who presented with fever, night sweats, weight loss, polyarthralgia, and bilateral hearing loss. Mediastinal and hilar lymphadenopathies with hypercaptation on positive emission tomography (PET) scans were present. Low-dose steroids were ineffective. She then developed bilateral anterior uveitis and right-sided peripheral facial palsy. Head magnetic resonance imaging (MRI) showed inflammatory involvement of the right cochlea, geniculate ganglion, and bilateral vestibulocochlear bundle. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Excisional biopsy of mediastinal lymph nodes confirmed the presence of noncaseating granulomas. The diagnosis of systemic sarcoidosis with serious neurological involvement was made and treatment with high-dose steroids led to significant clinical improvement. Sarcoidosis remains a diagnosis of exclusion based on supportive clinical, radiological, and histological findings. This case highlights the challenge it was to diagnose this disorder. Neurologic involvement in sarcoidosis is relatively uncommon and has an unpredictable clinical course and prognosis.
Acute generalized exanthematous pustulosis (AGEP) is a rare entity characterized by fever associated with the sudden appearance of erythematous lesions, on which multiple sterile, non-follicular pustules develop. We describe a case of a 44-year-old healthy male who developed fever and multiple erythematous and edematous lesions with progressive generalization to the entire body, associated with multiple small non-follicular pustules three days after having started flucloxacillin for the treatment of a furuncle. Considering the characteristics of the exanthema, fever, and association with aminopenicillin initiation, AGEP was considered. A skin biopsy revealed subcorneal and superficial epidermal pustules, with foci of spongiosis, papillary edema, and a superficial, perivascular inflammatory cell infiltrate with neutrophils and eosinophils, consistent with the clinical diagnosis of AGEP. The culprit drug was suspended, and prednisolone was started, considering the rash extension, with progressive and complete improvement. Although it is a rare condition, the hypothesis of AGEP should be considered in acute febrile conditions with disseminated pustules. It resolves spontaneously after discontinuation of the offending drug, and the diagnosis is based on clinical presentation and skin biopsy.
Introdução: A vídeocapilaroscopia periungueal é uma técnica não invasiva dirigida ao estudo da microcirculação. Este exame constitui-se como uma ferramenta adicional na avaliação do doente com alterações da microcirculação, na maioria dos casos por suspeita de doença autoimune. Métodos: Primeiros 101 doentes submetidos a vídeocapilaroscopia periungueal no nosso hospital num período de 18 meses, com recurso a um vídeocapilaroscópio digital. Resultados: Cento e um doentes, 85% mulheres, idade média 50 anos. O principal motivo de referenciação foi a existência de fenómeno de Raynaud (93%). A avaliação capilaroscópica foi sugestiva de fenómeno de Raynaud primário em 28 doentes e secundário em 65. Discussão: Os dados descritos, embora numa amostra ainda pequena, permitem reforçar a utilidade da vídeocapilaroscopia periungueal na avaliação inicial dos doentes com suspeita de disfunção da microcirculação, especialmente no contexto de patologia autoimune. A identificação precoce dos indivíduos com maior risco de progressão para doença autoimune permitirá otimizar o seu acompanhamento, nomeadamente com vigilância regular em consulta externa, facilidade de acesso à equipa médica assistente para deteção precoce de novos sintomas e avaliação laboratorial regular. Conclusão: A vídeocapilaroscopia periungueal é um método acessível e de baixo custo, que contribui conjuntamente com a avaliação laboratorial para a identificação precoce dos indivíduos com fenómeno de Raynaud secundário, podendo assim afetar positivamente o acompanhamento dos mesmos. O desenvolvimento futuro desta técnica poderá representar um preditor de progressão de doença.
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