Secondary Systemic Amyloidosis Research Articles

The heterogeneity of protein AA in secondary (reactive) systemic amyloidosis

In secondary systemtic amyloidosis, amyloid fibrils have protein AA as a main subunit protein. As judged from gel chromatography and electrophoresis, this protein is rather homogeneous. In the present paper it is shown, however, that protein AA is very heterogeneous and composed of many peptides with different isoelectric points. However, their antigenic properties and amino acid compositions vary only little. It is concluded that protein AA is as heterogeneous as its postulated precursor, the acute phase reactant serum AA and that a theory that only one or a few serum protein AA's can give rise to amyloid fibrils, might be wrong.

Localized Conjunctival Amyloidosis

We treated a patient with localized amyloidosis of the bulbar conjunctiva, which strongly resembled a neoplastic growth. Although the patient suffered from a long-standing, debilitating rheumatoid arthritis, suggesting the possibility of a secondary systemic amyloidosis caused by the chronic effusive process, no specific clinical evidence of systemic amyloidosis was noted. The ocular lesion was, therefore, classified as a primary localized amyloidosis. However, the simple recognition of amyloid in the ocular adnexa should not be considered a complete diagnosis. For such cases in which a prominent underlying systemic disease is present, a thorough medical evaluation and future follow-up should be ensured to rule out the possibility that this represents the initial manifestation of a potentially life-threatening secondary systemic amyloidosis caused by rheumatoid arthritis.

A New Method for the Diagnosis of Systemic Amyloidosis

Twenty-eight patients were clinically suspected of secondary amyloidosis; eight of them had abnormal results from fine-needle biopsy of subcutaneous abdominal fat. The abnormal results were later confirmed after rectal biopsy or autopsy. In addition, seven of the 19 patients with normal results also had normal results from rectal biopsies. Fine-needle biopsy of subcutaneous fat is a simple and reliable method for diagnosing secondary systemic amyloidosis.

Massive Bladder Hemorrhage after Cystoscopy in a Patient with Secondary Systemic Amyloidosis

Massive Bladder Hemorrhage after Cystoscopy in a Patient with Secondary Systemic Amyloidosis

OCCURRENCE OF AMYLOID DEPOSITS IN THE SKIN IN SECONDARY SYSTEMIC AMYLOIDOSIS

Among 9 patients with secondary systemic amyloidosis, 8 had amyloid deposits in the skin. Amyloid was found in the deepest part of the dermis around the adnexa and in the subcutaneous tissue in the form of rings around fat cells. The amount of amyloid varied somewhat between different skin areas and, among the parts of the body examined, the scalp and the abdominal skin showed the heaviest infiltration. It is obvious that secondary amyloidosis can be diagnosed by means of skin biopsy in many cases.

Secondary systemic amyloidosis: analysis of underlying disorders.

The authors have analyzed the disorders underlying the development of secondary amyloidosis in 100 cases. No unitary factor has been found to explain the development of secondary systemic amyloidosis.

The Cutaneous Amyloidoses

Histologic features of specimens of skin from 40 patients with systemic amyloidosis were correlated with the clinical findings. In primary systemic amyloidosis (including patients with myeloma) papules were correlated with amyloid deposits in dermal papillae, plaques with diffuse involvement of the corium, and nodules and tumefactions with infiltration of the reticular corium and subcutaneum. In patients with purpura, amyloid was deposited in the walls of dermal blood vessels. There was involvement of pilosebaceous units in all biopsy specimens from areas of alopecia. The electron microscopic appearance of the amyloid in skin in primary systemic amyloidosis was similar to that found in localized forms of cutaneous amyloidosis. Among 100 patients with secondary systemic amyloidosis who came to autopsy, no instance could be found of a clinical lesion caused by the deposition of amyloid in the skin. Among 60 clinical examples of cutaneous amyloidosis, no patient was found to have secondary systemic amyloidosis. Thus, when a dermatologic lesion is attributable to amyloid, an aggressive search for such conditions as tuberculosis is unwarranted, since secondary systemic amyloidosis would be a most unlikely cause. When a dermatosis is present in a patient with secondary systemic amyloidosis it may be related to the underlying disorder, but it is almost certainly not caused by amyloid.

Systemic Amyloidosis Complicating Dermatoses

Certain dermatoses may be complicated by systemic amyloidosis. The underlying disorders in eight of 100 patients with secondary systemic amyloidosis studied at autopsy were: hidradenitis suppurativa, stasis ulcers, psoriatic arthritis, basal cell carcinoma, dystrophic epidermolysis bullosa, and lepromatous leprosy (three cases). When proteinuria or hepatomegaly is discovered in a patient with a potentially amyloidogenic dermatosis, the possibility that secondary systemic amyloidosis has developed should be considered.

Five cases of lichen amyloidosis.

Amyloidosis of the skin is classified into the following 4 main groups: (1) primary systemic amyloidosis, systematized amyloidosis 1 ; (2) secondary systemic amyloidosis, generalized amyloidosis; (3) primary localized amyloidosis, lichen amyloidosis, or amyloidosis localis cutis nodularis et disseminata, 2-4 and (4) secondary localized amyloidosis (seniles Amyloid, Freudenthal 5 ). In primary systemic amyloidosis, the parenchyma of the organs is largely spared; blood vessels, connective tissues, and muscles are the main site of involvement. This type of amyloidosis is often accompanied with multiple myeloma, Bence Jones proteinuria, and plasmocytoma. Secondary systemic amyloidosis occurs in chronic diseases such as tuberculosis, leprosy, malaria, syphilis, osteomyelitis, and so on. Amyloid deposits are found in the parenchymatous organs, especially in the liver, kidney, spleen, and adrenals. In secondary localized amyloidosis, amyloid deposits are found in senile and pigmented verrucae, keratomas, epitheliomas, and cylindromas. Primary localized amyloidosis has also been called lichen amyloidosis. It is a very

Ocular manifestations of hereditary primary systemic amyloidosis.

Hereditary primary systemic amyloidosis, an unusual expression of the amyloid problem, is characterized by a protean symptomatology that remarkably mimics the commonplace and the obscure. To date only four reports have appeared in the world literature describing in some detail the clinical, genetic, and pathologic constellation.* Recently three reports have appeared which emphasize the remarkable involvement of the ocular apparatus.† As to the genesis of this universal and morbid process, much speculation has appeared in the literature in the past halfcentury. On the one hand, it has been held that the pathophysiology represents the endresult of an immunological reaction, and, on the other, it has been regarded as an anomaly of the connective tissue ground substance. For the greater part, these observations have been based upon, with their known limitation, fundamental animal experimental data. It has recently been suggested that secondary (parenchymatous) and primary (mesenchymal) systemic amyloidosis might represent similar

Amyloidosis cutis: a macular variant.

AMYLOID deposits are found in the skin in many diverse and unrelated conditions. The skin is rarely involved in the secondary systemic amyloidosis following chronic wasting diseases, but more often accompanies primary systemic amyloidosis with macroglossia. These comparatively rare conditions apparently have no connection with each other nor with the common localized amyloidosis cutis with which we are concerned in this report. First described as amyloidosis cutis nodularis et disseminata by Gutmann1in 1928 and as lichen amyloidosis by Freudenthal2in 1930, this disease has been the subject of many reports in this country and abroad. Several varieties have been reported. In all these cases the lichenoid character of the primary lesion has been emphasized. This primary lesion has been described by Andrews3as a small brownish, and slightly scaly papule which seems to be translucent and simulates a vesicle, but the fully developed papules are