A patient with a 10 year history of monoclonal gammopathy of undetermined significance and Bence Jones proteinuria, and a 44 year history of ankylosing spondylitis, developed a nephrotic syndrome secondary to renal amyloidosis. Clinically the amyloidosis was ascribed to Bence Jones proteinuria rather than to the burnt out ankylosing spondylitis. However, histochemical and immunofluorescence sta...

Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Lichen amyloidosis, a variant of localized cutaneous amyloidosis, is characterized by discrete, intensely pruritic, hyperkeratotic brown-coloured papules and plaques (1). Genetic and viral factors, as well as chronic friction due to scratching, are possible causes (2–4). Therapeutic management of lichen amyloidosis compr...

Amyloidosis is an uncommon disease that is characterized by abnormal extracellular deposition of misfolded protein fibrils leading to organ dysfunction. The deposited proteins display common chemical and histologic properties but can vary dramatically in their origin. Kidney disease is a common manifestation in patients with systemic amyloidosis with a number of amyloidogenic proteins discovere...

Many chronic inflammatory conditions can lead to systemic amyloidosis. However, secondary amyloidosis has rarely been associated with gout, and the literature reports only a handful of cases, all presenting with renal disease. We report a patient with a history of poorly controlled gout who presented with malabsorption. Endoscopic biopsies confirmed a diagnosis of small intestinal amyloidosis. ...

Introduction The most serious complication in Familial Mediterranean fever (FMF) is the development of amyloidosis, which usually determines the prognosis. Amyloid deposition can be systemic or organ-specific. The clinical features of amyloidosis are dependent on the organs involved, type of amyloidosis, rate of amyloid deposition and amount of amyloid fibrils. Organ dysfunction can cause life-...

Finnish-type familial amyloidosis (FAF) is a rare hereditary systemic amyloidosis that mainly exhibits cranial neuropathy. We describe a Japanese family with FAF manifested predominantly as renal amyloidosis. The proband was a 42-year-old woman with a 21-year history of proteinuria due to renal amyloidosis. Her mother was subsequently diagnosed with a similar disorder. After the first renal bio...