Macular amyloidosis is a major cause of skin pigmentation and a rare form of localized primary cutaneous amyloidosis in western countries, with a higher prevalence in Asia and South America. The etiopathogenesis of macular amyloidosis has not yet been fully clarified; a number of risk factors are involved, among them chronic friction in particular. A 54-year-old patient with macular amyloidosis...

Amyloidosis is characterized by a deposition of insoluble fibrils in various organs and tissues. Amyloid deposition, in the gastrointestinal track, provokes a dysfunction of the organ, due to an accumulation of fibrils, and causes a variety of clinical symptoms and endoscopic findings. Primary amyloidosis in the gastrointestinal tract is rarely reported in Korea. We experienced a case of recurr...

Amyloidosis is a disease related to abnormal protein folding and deposition of that abnormal protein between cells of the body in various tissues and organs, resulting in multiple clinical manifestations. We report a case of amyloidosis with atypical features, isolated to the mediastinum, in a 75-year-old male who presented with fatigue and shortness of breath. Amyloidosis that is isolated to t...

Amyloidosis is characterised by the extracellular deposition of abnormal protein fibrils. Recent advances in the study of amyloidosis have been based on the chemical analysis of these protein fibrils.1,2 This has also led to a more rational classification of the condition (Fig 1). Further discussion of localised forms of amyloidosis is outside the scope of this article, which will be confined t...

Background Amyloid cardiomyopathy is a polymorphic condition with heterogeneous prognosis. Whereas AL amyloid cardiomyopathy is the most frequent type of amyloid cardiopathy, transthyretin (TTR) amyloidosis is often under diagnosed. TTR gene sequencing may be easily performed although usually used after a histological proof of amyloidosis is obtained. We conducted a prospective study to evaluat...

Objective(s):Familial Mediterranean fever (FMF), an inherited autosomal recessive disorder, is frequently present among individuals of Mediterranean origin. Differences in the clinical manifestations of FMF between different ethnic groups have been documented. The aim of the present study was to determine the most common characteristics of FMF and the relationship between clinical findings and ...

BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...

A case of generalized amyloidosis associated with cyclic neutropenia is presented. A 24-yr-old female with cyclic neutropenia died from intestinal obstruction caused by necrosis and perforation of the small intestine. Post-mortem examination revealed generalized amyloidosis involving almost all organs. Amyloid deposits were prominent, especially in the alimentary tract, kidneys, spleen, and sma...

Urethral amyloidosis is a rare condition in which eosinophilic amyloid proteins are deposited in the urethra. Only a small number of reports on urethral amyloidosis have been published. Increased interest has been associated with this disease due to its clinical similarities with urothelial carcinoma. A biopsy of the lesion and a histological examination are essential for the correct diagnosis....

There is no specific therapy for primary amyloidosis, and acquired generalised amyloidosis can be treated only if the underlying disease is eliminated. In this study we have investigated the role of colchicine therapy in primary amyloidosis, and dimethylsulphoxide (DMSO) in leprosy associated secondary amyloidosis. No effect on creatinine clearance or 24 h proteinuria could be observed in the p...