Amyloidosis - Cause, Symptoms and Home Treatment of primary Amyloidosis

Amyloidosis results from the deposition of insoluble, fibrous amyloid proteins, mainly in the extracellular spaces of organs and tissues. Named by Virchow in 1854 on the basis of color after staining with iodine and sulfuric acid, all amyloid fibrils share an identical secondary structure, the b-pleated sheet conformation, and a unique ultrastructure. About 15 to 20% of patients with myeloma have amyloidosis.

Involvement of the skin is one of the most characteristic manifestations of primary (AL) amyloidosis. In AL amyloidosis, the usually nonpruritic lesions may consist of slightly raised, waxy papules or plaques that are usually clustered in the folds of the axillae, anal, or inguinal regions; the face and neck; or mucosal areas such as ear or tongue. Periorbital ecchymoses ("black eye" or "raccoon syndrome") have been reported.

Types of Amyloidosis

1. Systemic amyloidoses include biochemically distinct forms that are neoplastic, inflammatory, genetic, or iatrogenic in origin,
2. Localized or organ-limited amyloidoses are associated with aging and diabetes and occur in isolated organs, often endocrine, without evidence of systemic involvement.
3. Heredofamilial Amyloidoses - Heredofamilial amyloidoses other than the AA form associated with FMF and FHF primarily involve the nervous system, and their mode of inheritance is autosomal dominant.
4. Amyloid A Amyloidosis (AA) AA amyloidosis (secondary, reactive, or acquired amyloidosis) occurs most frequently as a complication of chronic inflammatory disease.
5. Light Chain Amyloidosis (AL) The most common form of systemic amyloidosis seen in current clinical practice is AL (primary idiopathic amyloidosis, or that associated with multiple myeloma) resulting from fibril formation by monoclonal antibody light chains in primary amyloidosis and in some cases of multiple myeloma.
6. Polypeptide Hormone-Derived Amyloidosis Amyloid deposits are common in polypeptide hormone-producing tissues and tumors.

Amyloidosis Associated with Alzheimer's Disease

A novel protein, b-amyloid protein (Ab), is the major fibril protein in the amyloid deposits of the cerebrovascular walls and the cores of the neuritic plaques of Alzheimer's disease (AD) patients and also in individuals with Down's syndrome. Mutations in AbPP are associated with familial AD and also with a different type of amyloidosis, hereditary cerebral hemorrhage with amyloidosis (Dutch type). Other forms of familial AD are associated with mutations in genes that encode presenilin proteins.

Diagnosis of Amyloidosis

The diagnosis of amyloidosis is usually not made until after the point of irreversible organ damage.The clinical manifestations of amyloidosis are varied and depend entirely on the biochemical nature of the fibril protein and thus the area of the body that is involved.

Treatment of Amyloidosis

Rational therapy should be directed at

(1) reducing precursor production,

(2) inhibiting the synthesis and extracellular deposition of amyloid fibrils, and

(3) promoting lysis or mobilization of existing amyloid deposits.

There are new specific therapies for the various amyloidoses.

Amyloidosis
Connective tissue naevi
Dermatofibroma
Dermatofibrosarcoma protuberans
Ehler's Danlos syndrome
Elastosis perforans serpiginosa
Keloids & hypertrophic scars
Lipodystrophy
Lipoma
Mastocytoma
Neurofibroma
Pseudoxanthoma elasticum
Reactive perforating collagenosis
Striae (stretch marks)
Eosinophilic fasciitis
Erythema multiforme
Histiocytoses
Jessner's lymphocytic infiltrate
Langerhans cell histiocytosis
Lymphocytoma cutis
Lymphomatoid papulosis
Mastocytosis
Morphoea
Non-Langerhans cell histiocytosis
Panniculitis
Reticulohistiocytosis
Sweet's disease
Systemic sclerosis
Atypical mycobacterial infection
Granuloma faciale
Granuloma annulare
Cutaneous tuberculosis
Leprosy
Pyogenic granuloma
Sarcoidosis