<\/span><\/h2>\nAmyloidosis is the term used for a group of diseases in which one or more organs in the body accumulate various insoluble proteins (amyloid<\/span>) in quantities to cause dysfunction<\/span> of the organ system. Organs often affected include the heart, kidneys, gastrointestinal tract, nervous system and skin. Amyloidosis of the skin is called cutaneous<\/span> amyloidosis. In this condition, amyloid or amyloid-like proteins are deposited in the dermis<\/span>.<\/p>\n<\/span>Types of amyloidosis<\/span><\/h2>\nThere are three major types of amyloidosis.<\/p>\n
Primary<\/span> amyloidosis <\/strong><\/p>\n\n- Primary amyloidosis is a protein disorder. metabolism<\/span> which originates in the bone marrow and is occasionally associated with multiple myeloma. It is also sometimes known as amyloid L (AL) chain type amyloidosis.<\/li>\n
- Primary systemic<\/span> Amyloidosis affects the heart, kidneys, liver, gastrointestinal tract, and central nervous system. Skin involvement occurs in approximately 30-40% of patients.<\/li>\n
- \nLocated<\/span> Primary amyloidosis affects specific organs such as the skin (primary cutaneous amyloidosis), heart, eyes, respiratory tract, and bladder.<\/li>\n<\/ul>\n
Types of primary localized cutaneous amyloidosis include:<\/p>\n
\n- Lichen amyloidosis<\/li>\n
- Macular<\/span> amyloidosis<\/li>\n
- \nNodular<\/span> amyloidosis<\/li>\n
- Amyloidosis skin<\/span> Dychromic.<\/li>\n<\/ul>\n
Secondary systemic amyloidosis. <\/strong><\/p>\n\n- Secondary systemic amyloidosis occurs as a complication of many chronic<\/span> inflammatory<\/span> diseases such as rheumatoid arthritis<\/span> and osteomyelitis<\/span>.<\/li>\n
- It is also known as amyloid A (AA) amyloidosis and is the most common form of systemic amyloidosis worldwide.<\/li>\n
- The kidney, liver and spleen are the organs most affected in secondary systemic amyloidosis. Skin involvement is rarely a feature of the disease.<\/li>\n<\/ul>\n
Family<\/span> (hereditary<\/span>) amyloidosis <\/strong><\/p>\nFamilial amyloidosis is a rare form of amyloidosis that is inherited and most often causes cardiomyopathy<\/span> and neuropathy<\/span> in middle age The most common form, known as ATTR amyloidosis, is due to a mutation<\/span> in transthyretin (TTR) gene<\/span> in chromosome<\/span> 5 and leads to defective hepatic<\/span> transthyretin protein.<\/p>\n\n
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<\/span>Clinical characteristics of primary amyloidosis.<\/span><\/h2>\nDistinctive clinical features are found for each type of primary amyloidosis.<\/p>\n
<\/span>Systemic amyloidosis<\/span><\/h3>\nSigns and symptoms of primary systemic amyloidosis are generally nonspecific and include:<\/p>\n
\n- Fatigue<\/li>\n
- Weightloss<\/li>\n
- Edema<\/span><\/li>\n
- Short of breath<\/li>\n
- Daze<\/li>\n
- Numbness, tingling sensation<\/li>\n
- Hoarseness.<\/li>\n<\/ul>\n
These can precede the diagnosis of amyloidosis by up to two years. However, when present along with the following specific symptoms, systemic amyloidosis should be considered a possible diagnosis.<\/p>\n
\n- Carpal tunnel syndrome<\/span>: Impaired function of the median nerve in the wrist as the nerve passes through the carpal tunnel.<\/li>\n
- Skin lesions\n
\n- The most common skin findings are purple<\/span>: petechiae<\/span> (small red blood stains) and ecchymosis<\/span> (small flat spots of blood that are round or irregular in shape and bluish\/purple).<\/li>\n
- Waxy papules<\/span>, nodules<\/span> or plates<\/span> can be found around the eyelids, neck, groin and anogenital<\/span> zone.<\/li>\n
- Blisters filled with blood may form, for example by pinching the skin.<\/li>\n
- the nail<\/span> can be brittle and brittle (clove dystrophy<\/span>)<\/li>\n
- Patches hair<\/span> a loss may arise (diffuse<\/span> alopecia<\/span>)<\/li>\n<\/ul>\n<\/li>\n
- Macroglossia: refers to an enlarged and firm tongue covered with blood spots, plaques and blisters.<\/li>\n
- Edema (tissue inflammation) develops as a result of heart failure or nephrotic syndrome (kidney disease).<\/li>\n
- \nHepatomegaly<\/span> (enlarged liver).<\/li>\n<\/ul>\n\n
<\/span>Systemic amyloidosis<\/span><\/h5>\n<\/section>\n\n\n
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Hereditary amyloid<\/p>\n
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Amyloidosis purpura<\/p>\n
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Amyloidosis purpura<\/p>\n
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Macrogloassia due to systemic amyloidosis<\/p>\n
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Macrogloassia due to systemic amyloidosis<\/p>\n
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Macrogloassia due to systemic amyloidosis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Lichen amyloidosis<\/span><\/h3>\n\n- Also called papular<\/span> amyloidosis<\/li>\n
- Lichen amyloidosis is the most common form of localized primary cutaneous amyloidosis.<\/li>\n
- It presents as intense itching. eruption<\/span> on the shins, thighs, feet and forearms.<\/li>\n
- The lesions consist of multiple raised spots (papules) that are scaly<\/span> and red\/brown.<\/li>\n
- The papules can get together<\/span> in thickened plates.<\/li>\n
- It appears to be more common in people of Chinese descent and men.<\/li>\n
- It occurs most frequently in people between 50 and 60 years of age.<\/li>\n
- It may be a variant of lichen simplex.<\/li>\n<\/ul>\n\n
<\/span>Lichen amyloidosis<\/span><\/h5>\n<\/section>\n\n\n
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Lichen amyloidosis<\/p>\n
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Lichen amyloidosis<\/p>\n
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Lichen amyloidosis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Macular amyloidosis<\/span><\/h3>\n\n- In macular amyloidosis, lesions appear as flat dark brown or grayish spots that may join together to form patches of dark skin.<\/li>\n
- The degree of itching varies from mild to severe.<\/li>\n
- The lesions are usually distributed symmetrically over the upper back between the shoulder blades, on the chest, and sometimes on the arms.<\/li>\n
- It appears to be more common in Asians, South Americans, and Eastern Orientals.<\/li>\n
- It usually presents in early adult life and affects women more often than men.<\/li>\n<\/ul>\n\n
<\/span>Macular amyloidosis<\/span><\/h5>\n<\/section>\n\n\n
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Macular amyloidosis<\/p>\n
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Macular amyloidosis<\/p>\n
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Macular amyloidosis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Nodular primary localized cutaneous amyloidosis<\/span><\/h3>\n\n- Also known as atrophic amyloidosis cutis nodularis and tumefactive amyloidosis<\/li>\n
- Nodular primary localized cutaneous amyloidosis is the rarest form of primary localized cutaneous amyloidosis.<\/li>\n
- Single or multiple firm nodules or plaques may appear on the trunk, extremities, extremities, face, and genitals.<\/li>\n
- Nodular primary localized cutaneous amyloidosis usually has no symptoms and patients seek medical attention for its appearance.<\/li>\n
- The nodules can be a few millimeters to a few centimeters and can be pinkish brown to red in color.<\/li>\n
- The lesions tend not to ulcerate, but some may crack or split, particularly those on the soles of the feet.<\/li>\n<\/ul>\n\n
<\/span>Nodular amyloidosis<\/span><\/h5>\n<\/section>\n\n\n
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Nodular amyloidosis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Amyloidosis cutis dyschromicum<\/span><\/h3>\nAmyloidosis skin<\/span> Dyschromia is a recently described form of primary cutaneous amyloidosis that causes localization hyperpigmentation<\/span> and hypopigmentation<\/span>.<\/p>\n<\/span>How is the diagnosis of amyloidosis made?<\/span><\/h2>\nThe diagnosis of the various types of primary cutaneous amyloidosis is made based on its clinical appearance and the characteristic histology<\/span> changes observed in the skin biopsy<\/span>. See also systemic amyloidosis. pathology<\/span>.<\/p>\n<\/span>What is the treatment of systemic amyloidosis?<\/span><\/h2>\n<\/span>Nonspecific treatment<\/span><\/h3>\nTreatment is aimed at maintaining the function of the affected organs. For example, kidney failure can be treated with dialysis<\/span> and congestive heart failure with diuretics.<\/p>\n<\/span>Specific treatment<\/span><\/h3>\n\n- \nChemotherapy<\/span> with melphalan and prednisone or dexamethasone (oral corticosteroids) may control<\/span> systemic amyloidosis<\/li>\n
- In secondary amyloidosis, colchicine may inhibit<\/span> amyloid statement<\/span> while the treatment is directed against the main cause.<\/li>\n
- Managed monoclonal<\/span> antibodies<\/span> in use for systemic amyloidosis include lenalidomide and bortezomib.<\/li>\n
- Bone marrow ablation<\/span> can be carried out to eliminate the plasma<\/span> cells and is followed by autologous stem cells transplant<\/span>.<\/li>\n
- Hereditary ATTR amyloidosis is treated with diflunisal or tafamidis (which stabilizes the abnormal transthyretin protein). Liver transplant is reserved for the most severely affected people.<\/li>\n<\/ul>\n