An adult male was described, who developed a tumour on his left arm, which was diagnosed as a primary cutaneous extramedullary plasmacytoma after histopathological, electron microscopic and immunocytochemical studies. Further studies ruled out the presence of multiple myeloma, extramedullary plasmacytoma of another site, or paraproteinaemia/paraproteinuria. During a relapse of the tumour 2 1/2 years after initial treatment, a new immunocytochemical study demonstrated that the tumour cells expressed monoclonal lambda IgG at the intracytoplasmic level, an unusual finding.
The Toxic Syndrome (TS) caused by ingestion of adulterated rapeseed oil in Spain is a new disease of multisystemic character whose aetiology and pathogenesis remains unknown. The most prominent pathological feature is a peculiar non-necrotizing vasculitis, that affects mainly the intima and involves vessels of every type and size in practically every organ. The TS begins with an acute clinical picture with pleuropneumopathy, fever, headaches, exanthems and eosinophilia. In these early clinical phases the main pathological findings were observed in the lungs and consisted of intense pulmonary interstitial oedema with scanty inflammatory mononuclear infiltrates. Ultrastructural study revealed hydropic degeneration of pneumocytes types I and II with desquamation of type I. The patients in this phase died of respiratory failure, later deaths were due to thromboembolic complications. Later still the patients developed a neuromuscular syndrome, sclerodermiform skin lesions and severe weight loss and died predominantly of infectious complications and respiratory failure. The anatomopathological picture in the peripheral nerves was that of inflammatory neuropathy with a lymphocytic perineuritis that led to perineural fibrosis with secondary axonal degeneration. The muscle presented an interstitial inflammatory myopathy at first followed by a neurogenic muscular atrophy. The skin lesions in the late phases consisted in dermal or dermal and subdermal fibrosclerosis, with vasculitis of the small arteries in the lower dermis. The salivary glands and pancreas showed vasculitis and interstitial inflammation which progressed to interstitial fibrosis and parenchymal atrophy.
In May 1981, a massive intoxication of people who had ingested adulterated cooking oil took place in Madrid and nearby provinces. Although the patients presented, in the first phase of the disease, with acute respiratory illness, later on thromboembolic complications, severe neuromuscular involvement, and scleroderma-like cutaneous lesions appeared. Kidneys were apparently spared; however, 4 out of 842 admitted patients developed glomerulonephritis; kidney biopsies revealed glomerular, vascular, tubular, and interstitial changes. Cases 1 and 3 had diffuse proliferative endocapillary glomerulonephritis; case 2 had diffuse membranoproliferative glomerulonephritis; and case 4 had diffuse extracapillary glomerulonephritis. Three cases had vascular lesions characterized by degenerative and proliferative endothelial changes, intimal foamy macrophages, and partial or complete obliteration of the vascular lumen by concentric myxoid fibrosis. There were signs of necrosis of tubular epithelium along with edema and lymphocytic and eosinophilic interstitial infiltration. Two out of 22 autopsies had segmentary glomerulonephritis, and 17 out of 22 autopsies showed renal vascular lesions.
Lymphomatoid papulosis (LyP) is a cutaneous eruption that is clinically benign but histologically malignant. To date, more than 300 cases have been published. About 10-20% of the patients develop a lymphoma. The purpose of this study was to make a clinicopathological study of 18 patients diagnosed with LyP in our hospital from 1973 to 1990, to characterize cellular infiltrates in the lesions, to find clonal populations of T-cells and to look for predictive factors of malignant lymphoma in LyP patients. Mean age was 48.7 years. The most frequent clinical lesions were papules (88.8%) followed by plaques (38.8%). The localizations were on extremities (loo%), trunk (88%), face (22%), palms or soles (ll%), perigenital(ll%) and scalp (5%). Two patients have been free of disease for more than 5 years. IgA levels are increased in LyP patients. Neither HTLV I nor 111 can be considered as a cause of the LyP in any of our patients. Associated diseases were found in 6 cases (1 mycosis fungoides, 1 Hodgkin's disease, 2 anaplastic large-cell lymphoma and 2 large plaque parapsoriasis). Some types of parapsoriasis should be included in the 'spectrum of Ki-1 lymphomas'. 52 skin biopsies were studied. 17% were type A of Willemze, 67% were type B and 15% were transitional. In 12 of the samples follicular or perifollicular infiltration was found. Follicular LyP should not be considered as a distinct type of LyP. Vasculitis is an uncommon finding in LyP. In all the cases studied, large atypical cells were CD30 + ; 5/7 cases had lost CD5 and 4/5 cases had lost CD7. In one case, all T-cell antigens were negative. Cerebriform mononuclear cells were always recognized by T-cell antibodies and they were CD30 positive in only two cases. In one case there were more Correspondence to: . 206 CD8 + than CD4 + cells. In 5 patients skin and blood samples for genetic rearrangement (beta-T) were taken. Only germinal line was found. We did not find any significant difference between those cases in which malignant lymphoma developed and those in which it did not.
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