Mutilating acral ulcers: the spectrum of differential diagnosis.

Prominent acral mutilating ulcers can be present in sensorimotor neuropathies. Although diabetes mellitus is the most common cause of neuropathic ulcers, these skin lesions may manifest in nondiabetic neuropathies. The dermatologic abnormalities may even precede the onset of typical neuropathic symptoms, leading to diagnostic confusion. Therefore, a broad differential diagnosis of neurological and systemic disorders should be considered when evaluating patients who have acral skin ulcerations. We report 3 cases of mutilating ulcers associated with nondiabetic neuropathies. The first case is a woman with multiple ulcerations on her forearm, hands, and toes. Her nerve biopsy revealed neuropathy with multiple congophilic deposits consistent with amyloid neuropathy. The second case is a woman with necrotic painless ulcer on her heel. Nerve biopsy in this patient revealed features suggestive of vasculitic neuropathy. The third case is a man with multiple ulcers on his extremities. A sural nerve biopsy in this patient was consistent with leprous neuropathy.

Doenças granulomatosas da laringe / Granulomatous diseases of the larynx

Realizamos uma revisäo sobre as principais doenças granulomatosas que podem acometer a laringe, devido à grande ocorrência dessas afecçöes no Brasil e em outros países de clima tropical e subtropical. Ressaltamos os aspectos histopatológicos para o seu diagnóstico, assim como o seu tratamento.

Renal involvement in leprosy.

Renal involvement in Hansen's disease was evaluated in 94 Portuguese patients, average age and duration of disease of 47.6 and 6.8 years respectively. Sixty-seven were studied retrospectively and 27 prospectively; renal biopsy was obtained in 4, fat-tissue needle aspiration for amyloidosis in 20, and tubular function was tested in ten. Mild proteinuria and/or haematuria was found in 33 patients, the severity increasing during erythema nodosum leprosum reactions, but without overt nephritic or nephrotic syndrome. Two patients had renal amyloidosis on biopsy and two more were confirmed by fat biopsy, a 10.5% incidence in those studied prospectively; all but one were of the lepromatous type, with frequent bouts of erythema nodosum leprosum. The two other renal biopsies showed mesangial glomerulonephritis, and one unexplained acute tubular necrosis; none had immune deposits by immunofluorescence. Proximal acidification was always normal, distal acidification tested by bicarbonate infusion was abnormal in one of nine patients, and six of nine patients had concentration defects. Leprosy causes frequent urinary sediment changes and concentration defects, usually without clinical expression; proteinuria and/or glomerular involvement is mainly due to amyloidosis.

Insuficiência hepática na hanseníase devida a amiloidose / Hepatic Insufficiency in leprosy had the amyloidosis

A case of hanseniasis with uncommonly severe secondary amyloidosis is reported and discussed. Clinical, laboratory and post-mortem findings show large involvement of the liver and hepatic deficiency, the principal cause of death. Hepatic deficiency with portal hypertension has been described in primary amyloidosis, but in the secondary type, as in this case, it has only been reported reported once, by Manzi et al (1971). It is attributed to protracted long term hanseniasis, hansenic reactions of the erythema nodosum type and osteomyelitis related to plantar ulcerations, all those conditions leading to an excess of antigenic stimulation and debilitation, of the humoral response. Althought 25 por cento of deaths due to hanseniasis in the West are caused by amuloidosis of the kidneys causing a nephrotic syndrome, in the case it did not lead to serious renal dysfunction

Amiloidosis secundaria en la lepra / Amyloidosis secondary leprosy

The incidence of secondary amyloidosis was studied in 306 leprous patients. According with the date encountered the following conclusion are drawn: 1- Lepromatous reaction is the condition more frecuently complicated by amyloidosis. 2- Lepromatous leprory "per se" in present times is a relatively rare cause (4,4%) occuring this complication in far avdance cases. 3- In the more benign forms (tuberculoid, etc) amyloidosis incidence is low occurred as results of complications of the disease