Amyloidosis is a rare disease that results from the extracellular deposition of amorphous fibrillar protein. It is usually observed in a systemic form. Gastrointestinal involvement is frequent but peritoneal localization is unusual. A 43-year-old male was investigated for nephritic colic. Morphologic explorations revealed small intestine agglomerans in the periumbilical region, infiltration of peritoneal fat and multiple coelio-mesenteric lymph nodes. There were no clinical or biological abnormalities and endoscopic examinations were normal. The patient then underwent an exploratory laparoscopy. Macroscopically false membranes were seen throughout the peritoneum and small bowel without ascites. Anatomopathologic examination diagnosed peritoneal amyloidosis. After several investigations a diagnosis of a primary peritoneal amyloidosis was confirmed. The patient was treated with melphalan and prednisone with a favorable outcome. Our case illustrates a particular presentation of peritoneal amyloidosis. Despite improved imaging methods, peritoneal biopsy remains essential for diagnosis.
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http://dx.doi.org/10.1007/s12328-011-0222-0 | DOI Listing |
J Clin Med
January 2025
Department of Pediatric Rheumatology, Institut de Recerca Sant Joan de Déu, Hospital Sant Joan de Déu, Universitat de Barcelona, 08950 Barcelona, Spain.
To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still's disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still's disease. This study included 107 patients (67% women), of whom 64 (59.
View Article and Find Full Text PDFClin Nucl Med
December 2024
From the Department of Nuclear Medicine, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey.
We present a case with systemic amyloidosis secondary to immunoglobulin light-chain amyloidosis (AL amyloidosis), which 18F-FDG PET/CT and 99mTc-PYP scintigraphy revealed amyloid deposition in the peritoneum, omentum, and mesentery. AL amyloidosis is characterized by the proliferation of clonal plasma cells and increased production and extracellular accumulation of immunoglobulin light chains, leading to organ malfunction. Even though AL amyloidosis can affect the gastrointestinal system, peritoneal involvement is rarely observed.
View Article and Find Full Text PDFCureus
September 2024
Center for General Medicine Education, School of Medicine, Keio University, Tokyo, JPN.
Familial Mediterranean fever (FMF) presents with various symptoms. Episodic abdominal pain is one of the most prevalent clinical characteristics of FMF and usually improves within 24-48 hours. We encountered a 50-year-old male patient from Japan who experienced recurrent episodes (several episodes occurring per year) of abdominal pain with fever since his late 20s.
View Article and Find Full Text PDFAutops Case Rep
September 2024
Universidade de São Paulo (USP), Faculdade de Medicina, Hospital das Clínicas, Serviço de Reumatologia, São Paulo, SP, Brasil.
Radiol Case Rep
November 2024
Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco.
AL amyloidosis is a rare systemic disease characterized by the deposition of amyloid protein in various organs, including the kidneys, heart, peripheral nervous system, digestive tract, skin, and muscles. Peritoneal involvement in AL amyloidosis is exceptionally rare. We present a unique case of AL amyloidosis with concurrent cardiac, cutaneous, and peritoneal manifestations.
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