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Anne de Muret

Publications and source records attributed to Anne de Muret.

10 recordsLinked to original sources
PubMed2006

[Adult idiopathic neutrophilic eccrine hidradenitis treated with colchicine].

INTRODUCTION: Neutrophilic eccrine hidradenitis (NEH) is a rare disease belonging to the group of neutrophilic dermatoses. It has a characteristic histopathologic pattern, with necrosis of the eccrine glands and a local neutrophilic infiltrate. It occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia or, less frequently, another malignancy. Its occurrence in healthy patients is rare. CASE REPORT: We report the case of a 56-year-old woman with no remarkable medical history who developed an erythematous papular facial eruption. Skin biopsy showed typical features of neutrophilic eccrine hidradenitis including neutrophilic infiltrate. No cause was found. The patient was successfully treated with colchicine. DISCUSSION: This case is particular because NEH was not associated with malignant hematologic disease, solid cancer, chemotherapy, fever or any other disease, after a follow-up of 22 months. Such idiopathic NEH has been reported only rarely in adults. The second particularity is the length of the eruption, which required colchicine treatment. Clinical improvement occurred within 1 month. Because hematologic malignancies can in some cases be preceded by neutrophilic dermatitis, clinical follow-up is recommended in adults.

Colchicine↗
PubMed2006

Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal hepatic fibrosis.

Non-cirrhotic perisinusoidal hepatic fibrosis is a process of imprecise pathogenesis involving collagenization of the space of Disse. Exposure to chemicals, auto-immunity, thrombophilia and/or infections are suspected primary agents. Here, we present the case of a patient who developed severe portal hypertension with histological features suggesting a non-cirrhotic perisinusoidal hepatic fibrosis. A 52-year-old man was hospitalized for oesophageal variceal haemorrhage. Liver cirrhosis or portal vein thrombosis were absent as attested by laboratory tests, duplex sonography, computed tomography scan and histological examination of a liver biopsy specimen. Presinusoidal portal hypertension was suggested by a normal wedge-free hepatic vein gradient. Only electron microscopy examination of a liver biopsy specimen could disclose perisinusoidal fibrosis. This was most probably secondary to a combined chemotherapy received 4 years earlier for non-Hodgkin large-cell lymphoma. As variceal ligation failed to control oesophageal varices while liver function tests were normal, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. This dramatically improved the signs of portal hypertension. This case illustrates the use of TIPS in the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal fibrosis.

Esophageal and Gastric Varices↗
PubMed2004

[Hematodermic CD4/CD56 neoplasm].

Hematodermic CD4/CD56 neoplasm is a recently described entity. This name has been initially proposed by the French Study Group on Cutaneous Lymphomas which established the primary anatomoclinical and pathogenic and cytogenic bases of the disease in 1999. This descriptive and provisional name allowed conceptualizing the entity by its main clinical and phenotypical characteristics. The first case in the literature goes back to 1994. Since that time, several other cases have been published. The expression of CD56 led most of the authors to propose an NK-cell lineage origin. In the last WHO classification of lymphomas, the entity was indexed under the name of "blastic NK-cell lymphoma". However, the authors underlined that there were currently no clues to the etiology of blastic NK-cell lymphoma and that the precise lineage of this disease was still unresolved. At the clinical level the main characteristics of the disease are the skin tropism and the occurrence of a leukemic phase at any time during the course of the disease. The median age is 59 but pediatric cases do exist. At the morphological level skin biopsy shows a monomorphous cell proliferation simulating a pleomorphic T cell cutaneous lymphoma. The diagnosis is based on phenotypic criteria which require frozen tissue. Currently, the main characteristics are the expression of CD4 and CD56 antigens while the main defined lineage specific markers are negative (B-cell, T-cell, NK-cell and myeloid-cell lineages). The origin of the tumor cells still remains uncertain but the plasmacytoid dendritic cell is presently a very serious candidate. The tumor cells share a great phenotypical homology and particularly the expression of the CD123 antigen. Functional homologies have also been demonstrated with tumor cells in vitro. Outcome of CD4/CD56 hematodermic neoplasms is very bad. The median time of survival is 14 months irrespective of the treatment given. Conventional chemotherapies used for the treatment of aggressive lymphomas or acute myeloid leukemias are quickly inefficient.

Adolescent↗
PubMed2003

Familial liver adenomatosis associated with hepatocyte nuclear factor 1alpha inactivation.

BACKGROUND & AIMS: Germline mutations in hepatocyte nuclear factor 1alpha (TCF1/HNF-1alpha) are associated with maturity-onset diabetes of the young type 3 (MODY3), and somatic biallelic inactivations of the gene are found in hepatocellular adenomas and liver adenomatosis. This study investigated cosegregation of HNF-1alpha germline mutations with diabetes and liver adenomatosis in 2 families. METHODS: Two unrelated patients with liver adenomatosis and harboring HNF-1alpha germline and somatic mutations were studied. Subsequently, we screened 9 relatives in the 2 independent families for diabetes, hepatocellular adenomas, and HNF-1alpha germline mutations. RESULTS: In family A, a father and his son presented with an intraperitoneal hemorrhagic rupture of a liver adenomatosis without diabetes. A heterozygous R229X germline mutation was identified in HNF-1alpha in the father and his son and also in his second 27-year-old son without hepatocellular adenomas. In family B, a diagnosis of liver adenomatosis was made fortuitously in a 14-year-old girl. A heterozygous G55fsX57 germline mutation in HNF-1alpha was identified in this patient, her diabetic father, and her 2 sisters. Systematic exploration showed liver adenomatosis in the 2 sisters. Somatic inactivation of the second HNF-1alpha allele was found in liver tumors in both families. CONCLUSIONS: This study describes familial liver adenomatosis and shows the association with germline HNF-1alpha mutations in adults and children. It also highlights the importance of screening for hepatocellular adenomas, diabetes, and HNF-1alpha germline mutations in relatives of patients with liver adenomatosis. Finally, prevalence of liver adenomatosis remains to be evaluated in MODY3 subjects.

Adenoma↗
PubMed2003

[Recurrent glucagonoma 20 years after surgical resection].

Glucagonoma is a rare islet alpha-cell pancreatic tumor. Patients usually present with necrolytic migratory erythema, diabetes mellitus, thromboembolism, and weight loss. Diagnosis is based on the presence of a pancreatic tumor in association with hyperglucagonemia. Tumor characterization is made by computed tomography and/or pancreatic endoscopic ultrasonic and indium-labeled octreo-scan. Surgery is the main component of the treatment, in some cases in association with chemotherapy. We report the case of a 72-year-old patient who developed a recurrent glucagonoma, 20 years after surgical resection.

Aged↗