Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Abstract: Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) / Reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS)

SUMMARY Objective: To review the hypersensitivity reaction to drugs known as drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), based on a case report. We also intend to discuss the difficulty and importance of disease recognition, since none of the changes is pathognomonic of this disease and failure to identify it may have disastrous consequences for the patient. Method: To describe this case report, in addition to the information collected for clinical assessment, a literature review was performed in the PubMed and Bireme databases in order to retrieve the latest information published in literature on DRESS syndrome. Results: The case of a 20-year old patient is reported. After anamnesis, physical examination and laboratory tests a diagnosis of DRESS syndrome was performed, characterized by rash, hematologic alterations, lymphadenopathy and lesions in target organ. This is a rare syndrome, whose frequency varies according to the drug used and the immune status of the patient, being more often associated with the use of anticonvulsants. Conclusion: The approach and discussion of the topic are of paramount importance, in view of the potential lethality of this treatable syndrome. Recognizing the occurrence of DRESS syndrome and starting treatment as soon as possible is crucial to reduce the risk of mortality and improve prognosis.

RESUMO Objetivo: fazer uma revisão da reação de hipersensibilidade a drogas denominada reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS), com base em um relato de caso clínico. Pretende-se ainda discutir a dificuldade e importância de seu reconhecimento, uma vez que nenhuma alteração dessa doença é patognomônica e sua não identificação pode trazer consequências desastrosas para o paciente. Método: para descrever este relato, além das informações coletadas no caso clínico, uma revisão da literatura nas bases de dados PubMed e Bireme foi realizada com o intuito de rever as informações mais recentes publicadas na literatura acerca da síndrome DRESS. Resultados: relatou-se o caso de uma paciente de 20 anos de idade que, após anamnese, exame físico e exames laboratoriais, foi diagnosticada com síndrome DRESS, caracterizada por erupção cutânea, alterações hematológicas, linfonodomegalia e lesões em órgão-alvo. É uma síndrome rara, cuja frequência varia conforme a droga utilizada e o estado imunológico do indivíduo, sendo mais associada ao uso de anticonvulsivantes. Conclusão: a abordagem e discussão do tema são de extrema importância, tendo em vista o potencial de letalidade dessa síndrome, que possui tratamento. Reconhecer precocemente a DRESS e instituir terapêutica é fundamental para reduzir o risco de mortalidade e melhorar o prognóstico.

Hypoxia/reoxygenation-induced myocardial lesions in newborn piglets are related to interindividual variability and not to oxygen concentration

OBJECTIVE: Evaluation of myocardial histological changes in an experimental animal model of neonatal hypoxiareoxygenation. METHODS: Normocapnic hypoxia was induced in 40 male Landrace/Large White piglets. Reoxygenation was initiated when the animals developed bradycardia (HR <60 beats/min) or severe hypotension (MAP <15 mmHg). The animals were divided into four groups based on the oxygen (O2) concentration used for reoxygenation; groups 1, 2, 3, and 4 received 18%, 21%, 40%, and 100% O2, respectively. The animals were further classified into five groups based on the time required for reoxygenation: A: fast recovery (<15 min); B: medium recovery (15-45 min); C: slow recovery (45-90 min); D: very slow recovery (>90 min), and E: nine deceased piglets. RESULTS: Histology revealed changes in all heart specimens. Interstitial edema, a wavy arrangement, hypereosinophilia and coagulative necrosis of cardiomyocytes were observed frequently. No differences in the incidence of changes were observed among groups 1-4, whereas marked differences regarding the frequency and the degree of changes were found among groups A-E. Coagulative necrosis was correlated with increased recovery time: this condition was detected post-asphyxia in 14%, 57%, and 100% of piglets with fast, medium, and slow or very slow recovery rates, respectively. CONCLUSIONS: The significant myocardial histological changes observed suggest that this experimental model might be a reliable model for investigating human neonatal cardiac hypoxia-related injury. No correlation was observed between the severity of histological changes and the fiO2 used during reoxygenation. Severe myocardial changes correlated strictly with recovery time, suggesting an unreported individual susceptibility of myocardiocytes to hypoxia, possibly leading to death after the typical time-sequence of events.

Hypereosinophilic syndrome

Hypereosinophilic syndrome is a rare disease with variable outcome. It is usually a disease of young adults. Involvement of the heart and or lungs occurs in 40-60% of cases which can lead to severe heart and lung failure. Direct tissue eosinophilic infiltration and release of their toxic granules are the main underlying pathology. In this study, we report a young lady presented with a short history of fever, easy fatigability, weight loss and bilateral lung infiltrates. The diagnosis was consistent with Hypereosinophilic syndrome and despite treatment with pulses of intravenous Methylprednisolone, oral Prednisolone, Hydroxyurea and Imitinab Mesylate, the patient unfortunately died because of severe respiratory failure. The age group, striking eosinophilia and fulminant fatal course without neoplasia warranted reporting

Factor V Leiden mutation and deep venous thrombosis in a patient with hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome is a rare condition characterized by extremely high peripheral blood eosinophil counts. Patients with idiopathic hypereosinophilic syndrome are at increased risk for thrombosis. The coexistence of idiopathic hypereosinophilic syndrome with other thrombotic disease is rare. We present an additional case of idiopathic hypereosinophilic syndrome and factor V Leiden mutation, which lead to deep vein thrombosis

Síndrome hipereosinofílica idiopática associada à doença eosinofílica disseminada em um cão / Hypereosinophilic idiopathic syndrome associated with disseminated eosinophilic disease in a dog

Relata-se um caso de síndrome hipereosinofílica idiopática associada à doença eosinofílica disseminada em um cão, macho, mestiço Pastor Alemão, com cinco anos de idade. Os sinais clínicos incluíam apatia, anorexia, intolerância ao exercício, caquexia, dispnéia e taquicardia. Laboratorialmente, havia reação leucemóide eosinofílica e na citologia da medula óssea observou-se acentuada hiperplasia eosinofílica. Radiologicamente, detectou-se uma área radiopaca intratorácica bilateral cranial ao coração. A punção aspirativa intratorácica demonstrou grande quantidade de eosinófilos, o que permitiu um diagnóstico clínico de infiltração pulmonar com eosinofilia. O diagnóstico foi confirmado histologicamente.

Neuropatia periférica e miosite na síndrome hipereosinofílica idiopática: relato de caso / Peripheral neuropathy and myositis in idiopathic hypereosinophilic syndrome: case report

Descrevemos um caso de síndrome hipereosinofílica idiopática, com manifestações clínicas de neuropatia periférica e sinais de miosite inflamatória. Trata-se de mulher de 20 anos de idade, que apresentou dificuldade progressiva para caminhar com quedas freqüentes e edema de membros inferiores até o nível do joelho, associado a parestesias e cãibras. O exame neurológico revelou hipotonia, arreflexia e redução da força e sensibilidade nos membros inferiores. O exame parasitológico de fezes foi negativo e o hemograma mostrou 24 por cento de eosinófilos (1848/mm ). Estudo eletrodiagnóstico mostrou comprometimento axonal sensitivo-motor nos nervos dos membros inferiores. A biópsia muscular mostrou discreta reação inflamatória perivascular e intersticial. Tratada com prednisona a paciente apresentou remissão dos sintomas em dois meses.

Hypereosinophilic Syndrome : CT Findings in Patients with Hepatic Lobar or Segmental Involvement

OBJECTIVE: The purpose of this study was to describe the CT findings of hepatic hypereosinophilic syndrome in which hepatic lobes or segments were involved. MATERIALS AND METHODS: Seven patients with hypereosinophilic syndrome with hepatic lobar or segmental involvement were included in our study. In all seven, diagnosis was based on liver biopsy and the results of corticosteroid treatment. CT findings were retrospectively reviewed by three radiologists, who reached a consensus. Biopsy specimens were examined, with special reference to portal and periportal inflammation. RESULTS: CT demonstrated well-defined, homogeneous or heterogeneous low attenuation with a straight margin limited to a hepatic lobe (n = 2), segments (n = 3), or subsegments (n = 2), particularly during the portal phase. Where there was subsegmental involvement, lesions were multiple, ovoid or wedge-shaped, and showed low attenuation. In two patients with lobar or segmental involvement, segmental portal vein narrowing was observed. Histopathologic examination dis-closed eosinophilic infiltration in the periportal area, sinusoids and central veins, as well as portal phlebitis. CONCLUSION: Hypereosinophilic syndrome may involve the presence of hepatic lobar, segmental, or subsegmental low-attenuated lesions, as seen on CT images. Their presence may be related to damage of the liver parenchyma and to portal phlebitis.

Hypereosinophilic Syndrome : CT Findings in Patients with Hepatic Lobar or Segmental Involvement

OBJECTIVE: The purpose of this study was to describe the CT findings of hepatic hypereosinophilic syndrome in which hepatic lobes or segments were involved. MATERIALS AND METHODS: Seven patients with hypereosinophilic syndrome with hepatic lobar or segmental involvement were included in our study. In all seven, diagnosis was based on liver biopsy and the results of corticosteroid treatment. CT findings were retrospectively reviewed by three radiologists, who reached a consensus. Biopsy specimens were examined, with special reference to portal and periportal inflammation. RESULTS: CT demonstrated well-defined, homogeneous or heterogeneous low attenuation with a straight margin limited to a hepatic lobe (n = 2), segments (n = 3), or subsegments (n = 2), particularly during the portal phase. Where there was subsegmental involvement, lesions were multiple, ovoid or wedge-shaped, and showed low attenuation. In two patients with lobar or segmental involvement, segmental portal vein narrowing was observed. Histopathologic examination dis-closed eosinophilic infiltration in the periportal area, sinusoids and central veins, as well as portal phlebitis. CONCLUSION: Hypereosinophilic syndrome may involve the presence of hepatic lobar, segmental, or subsegmental low-attenuated lesions, as seen on CT images. Their presence may be related to damage of the liver parenchyma and to portal phlebitis.