Lichen amyloidosus: a study of clinical, histopathologic and immunofluorescence findings in 30 cases.

BACKGROUND: Lichen amyloidosus (LA) is a primary localized cutaneous amyloidosis characterized clinically by discrete hyperkeratotic hyperpigmented papules and histologically by deposition of amyloid material in previously normal skin without any evidence of visceral involvement. AIMS AND OBJECTIVES: The aim of this work was to study the etiology, clinical features, histopathology and direct immunofluorescence findings in LA. METHODS: A prospective study of 30 patients with clinical, histological and immunofluorescence findings suggestive of LA was undertaken. After a detailed history and clinical examination, two punch biopsies for histopathology and immunofluorescence were taken. RESULTS: Of the 30 patients, 19 (63.3%) were males and 11 (36.7%) were females with duration of LA ranging from 6-20 months. Pruritus was the presenting symptom in 27 (90%) patients. Shin was involved in 26 (86.7%) followed by arms in three (10%) and back in one (3.3%). Seventeen patients (56%) had used scrubs for more than 2 years. Histopathology, direct immunofluorescence and Congo red staining detected amyloid in all cases. CONCLUSIONS: LA commonly presents over the shins as pruritic discrete hyperpigmented papules. Familial predisposition and friction may have a pathogenic role. Histopathological examination is very useful in the detection of amyloid which may be supplemented with direct immunofluorescence and Congo red staining.

Changing patterns of disease in Papua New Guinea over a period of 40 years from 1962. Pathology encountered in a stoneage culture by the first western-trained doctors who entered the country.

The diseases observed in Papua New Guinea coastal and highland people were described: segmental enteritis necroticans caused by the ingestion of pig meat contaminated with Clostridium welchii type C; chest empyemas caused by thoracostomies performed by village doctors because of ancient superstition; traumatic injuries; primary and secondary (lepromatous leprosy or tuberculosis) amylosis; slow prominent atherosclerosis and myocardial infarction.

The pattern of amyloidosis in Malaysia.

Congo red screening of routine biopsies at the University Hospital Kuala Lumpur revealed the following categories of amyloidosis: systemic AL (5.9%); systemic AA (3.2%); isolated atrial (14%); primary localized cutaneous (7.5%); other primary localized deposits (3.2%); localized intratumour (58%); and dystrophic (8.6%). Unlike in the West, AA amyloidosis in this population was usually secondary to leprosy or tuberculosis. Liver involvement in AL amyloidosis was shown to exhibit a sinusoidal pattern and differed from the vascular pattern of AA amyloidosis. Within the category of AA amyloidosis, there were two patterns of renal involvement--glomerular and vascular, with the glomerular pattern carrying a more ominous clinical picture. Notable among the localized amyloidoses were isolated atrial amyloidosis complicating chronic rheumatic heart disease, intratumour amyloidosis within nasopharyngeal carcinomas and dystrophic amyloidosis which occurred in fibrotic tissues.

The pattern of amyloidosis in a Malaysian patient population.

Congo red screening of 27,052 routine biopsy specimens from 22,827 patients over a 5 1/2-year period in the Department of Pathology, University of Malaya detected 186 cases of amyloidosis. The categories of amyloidosis encountered and their prevalences in relation to each other were: systemic AL (5.9%); systemic AA (3.2%); isolated atrial (14%); primary localized cutaneous (7.5%); other primary localized deposits (3.2%); localized intratumour (58%); and dystrophic (8.6%). A third of patients with systemic AL amyloidosis had coexistent immunocyte abnormality. The commonest underlying pathology for systemic AA amyloidosis was leprosy. Notable among the types of localized amyloidosis revealed by this study were isolated atrial amyloidosis, which appeared to complicate chronic rheumatic heart disease, and intratumour amyloidosis complicating nasopharyngeal carcinoma. Other tumours in which amyloid deposits were observed included basal cell carcinoma, islet cell tumour and medullary carcinoma of the thyroid. Dystrophic amyloidosis was observed in fibrotic tissues, such as damaged cardiac valves and osteoarthritic joints. Heredofamilial amyloidosis, senile systemic amyloidosis and degenerative cerebral amyloidosis were notably absent from this study.

An immunohistochemical and morphological study of amyloidosis complicating leprosy in Malaysian patients.

Congo red screening of tissue blocks from 37 consecutive autopsies on leprosy patients revealed 7 cases of systemic amyloidosis, indicating a prevalence rate of 19%. 5 were males and 2 females. All were ethnic Chinese. Their ages ranged from 52 to 85 years with a mean of 69 years. Six had lepromatous leprosy while the remaining 1 had tuberculoid leprosy. In all 7 cases, the amyloid was AA in type, being permanganate-sensitive and immunoreactive with anti-human AA protein antiserum. Hepatic deposition was limited to blood vessels, a pattern typical of AA (secondary) amyloidosis. With regard to renal involvement, 4 showed a predominantly vascular pattern of infiltration while 3 exhibited the more ominous glomerular pattern. Three died of chronic renal failure and 2 of congestive cardiac failure attributable to renal and cardiac amyloidosis respectively. One patient succumbed to septicaemia and the remaining 1 to acute myocardial infarction. AA amyloidosis remains a serious and significant complication of leprosy among Malaysians.

Pattern of renal amyloidosis in Indian patients.

Two hundred and thirty-three patients with renal amyloidosis were studied in an attempt to identify the incidence and pattern of the disease in northern India. The incidence of amyloidosis was 1.01% of 6431 post-mortems and 8.4% of 1980 renal biopsies from patients who presented with clinical evidence of glomerular disease. Two hundred and three patients (87.1%) had secondary amyloidosis, 22 (9.4%) had primary amyloid and 8 patients (3.5%) had amyloidosis associated with multiple myeloma. Tuberculosis of various organs was the commonest predisposing disease accounting for 59.1% of secondary amyloidosis, followed by chronic suppurative lung disease in 24.1%. Rheumatoid arthritis, chronic osteomyelitis and lepromatous leprosy were seen in a small percentage of patients (2 to 8%). Proteinuria of varying degree was present in all the 233 patients and 12.9% of them had a daily protein excretion of more than 10 g. Post-mortem examination of 65 patients with renal amyloidosis showed that 75.3% also had amyloid deposit in the spleen, 63% in the liver, and 50.8% in the adrenals. Clinical evidence of disappearance of proteinuria was observed in 3 patients with secondary amyloidosis; in 2 of them, the regression of amyloidosis was confirmed by serial renal biopsy performed 3 and 5 years after the initial diagnosis.

Amyloidosis in leprosy.

Fourteen hundred and forty five biopsied tissues of various organs including liver, skeletal muscle, kidney, lymphnode, larynx and skin from 1222 patients of leprosy obtained during the last 10 years have been examined histopathologically to study various types of pathological lesions. These patients ranged in age from 7 years to 72 years and the duration of illness varied from less than one year to twenty years. Although the presence of lepromatous granuloma, AFB in varying proportions, and some nonspecific lesions have been observed in them, but amyloidosis was not detected in any of them. This finding is discussed in view of the observations of other authors of different parts of the world in this connection. Consumption of mainly vegetarian diet in our population and that of meat in Western population has been suggested to be the probable cause of the difference of amyloidosis observed in the two groups of people.

Amyloidosis in leprosy

Fourteen hundred and forty five biopsied tissues of various organs including liver, skeletal muscle, kidney, lymphnode, larynx and skin from 1222 patients of leprosy obtained during the last 10 years have been examined histopathologically to study various types of pathological lesions. These patients ranged in age from 7 years to 72 years and the duration of illness varied from less than one year to twenty years. Although the presence of lepromatous granuloma, AFB in varying proportions, and some nonspecific lesions have been observed in them, but amyloidosis was not detected in any of them. This finding is discussed in view of the observations of other authors of different parts of the world in this connection. Consumption of mainly vegetarian diet in our population and that of meat in Western population has been suggested to be the probable cause of the difference of amyloidosis observed in the two groups of people.

Geographical distribution of amyloidosis in Papua New Guinea.

During an 11 year period 73 cases of amyloidosis were diagnosed at Port Moresby General Hospital as a result of histological examination of autopsy or biopsy material. No case of amyloidosis came from either the Highlands or North Coast regions although these two areas together accounted for 16% of the medical admissions at the hospital and made up a similar percentage of Port Moresby's Papua New Guinean population. Within the sub-district in which Port Moresby is situated there was a much higher incidence of amyloidosis in the inland, more sparsely populated, census divisions of the sub-district. Aetiological factors which may be related to the common occurrence of amyloidosis in Papua New Guinea are discussed in light of the observed geographical distribution of cases.

Secondary amyloidosis in lepromatous leprosy: possible relationships of diet and environment / ?

Some of the possible factors involved in the genesis of amyloidosis during the course of lepromatous leprosy have been studied. In a group of 101 patients from the U. S. Public Health Service Hospital at Carville, La., it is estimated that 40 to 50% have concomitant amyloidosis. This high proportion has been previously wll-documented by autopsy studies (2,3) and was suggested by a 31% positive result in 69 gingival biopsies among this group. Congo red results were often positive, particularly when accompanied by persistent albuminuria. The high percentage of amyloidosis at Carville lies in distict contrast to that of 119 patients studied at Guadalajara, Mexico, where amyloid was diagnosed in only 6%. Factors which may play some role in this different include discrepancies in animal fat consumption between the two population groups. Patients at Carville eat fewer calories but twice the percentage of saturated fat in their diet as compared to the Mexican group studied...