Amyloidosis is a rare metabolic disease associated with the formation of an abnormal amyloid protein and has complex mechanism development in which hereditary, endocrine, enzyme immune factors are involved. There several types amyloid, main AL derivative light chains immunoglobulins, AA acute phase -globulin keratin AK. can be systemic, damage to various organs tissues localized (local). With a...

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. Its cause is unknown. Five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associa...

Transthyretin amyloidosis is a conformational pathology characterized by the extracellular formation of amyloid deposits and the progressive impairment of the peripheral nervous system. Point mutations in this tetrameric plasma protein decrease its stability and are linked to disease onset and progression. Since non-mutated transthyretin also forms amyloid in systemic senile amyloidosis and som...

BACKGROUND Primary localized cutaneous amyloidosis (PLCA) is a chronic pruritic dermatological disorder of unknown aetiology. Genetic mutations in cases of familial PLCA have been mapped to the oncostatin-M receptor (OSMR) β, a subunit of interleukin (IL)-31 receptor. IL-31 has been implicated in the pathogenesis of atopic dermatitis (AD). OBJECTIVES To assess if AD is more prevalent in patie...

Primary cutaneous marginal zone B-cell lymphoma (MZL) is considered a cutaneous counterpart of extranodal MZL of mucosa-associated lymphoid tissue and an indolent lymphoma. Amyloid deposition in this tumor is very rare. We report here a case of primary cutaneous MZL with massive amyloid deposition. The tumor disseminated to the dura and mimicked primary dural MZL. The patient had a four year hi...

Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome, also called Neonatal Onset Multisystemic Inflammatory Disease (NOMID) is characterised by the triad of cutaneous rash, chronic meningitis and arthropathy. It is a chronic inflammatory illness that starts most often at birth and persists for the whole lifespan of the patient. Attempts at therapy have been disappointing. The...

A 79-year-old man presented with a large tumor on the left side of his head, which had grown over 5 years. Regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration. Histology showed that the entire dermis was filled with amorphous eosinophilic material. Immunohistochemistry was negative for cytokeratin, but showed that the dermis and parts of the subcu...

Published: J Turk Acad Dermatol 2016; 10 (2): 16102l1. This article is available from: http://www.jtad.org/2016/2/jtad16102l1.pdf