[Mycosis fungoides with epithelioid granulomas].
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Biomedical subjects
Publications and source records attributed to F Cottenot.
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A patient with subcorneal pustular dermatosis was found to have a circulating monoclonal IgA kappa immunoglobulin. Direct immunofluorescence studies revealed IgA kappa deposits in the subcorneal zone of the epidermis. Circulating IgA kappa reacting with the subcorneal zone of normal human epidermis was demonstrated by indirect immunofluoresence. It is speculated that IgA deposition might be implicated in the pathogenesis of subcorneal pustular dermatosis.
A 2-month, double-blind, placebo-controlled clinical trial was undertaken to evaluate the effectiveness of cimetidine in the treatment of chronic, plaque-type psoriasis. 24 patients completed the trial (11 treated with cimetidine, 13 with placebo). 3 patients in the cimetidine group and 10 in the placebo group improved. This difference is significant (p less than 0.01). Cimetidine had to be discontinued in 2 patients because of side effects. In this study, cimetidine proved of no benefit in the treatment of psoriasis. Furthermore, our results suggest the possibility of an adverse effect of cimetidine in psoriasis.
Circulating T cells, T helper, and T suppressor cells were investigated in 24 lepromatous patients, using murine hybridoma-derived monoclonal antibodies OKT 3, OKT 4, and OKT 8. Six bacillary lepromatous patients without recent eNL were studied; in this group, suppressor cells were increased and helper cells diminished, resulting in a decrease in the helper/suppressor (H/S) ratio. Nine bacteriologically negative lepromatous patients without recent ENL were studied. T cell subsets distribution was normal, although some T cell functions were affected. It was further shown that in non-ENL patients, the diminution of the H/S ratio is correlated with the Bacteriologic Index (BI). Although bacillary, ENL patients exhibit a completely different T cell pattern than non-reactional patients. In these patients, there was a significant diminution of circulating suppressor cells, and an increase in T cell functions. These abnormalities were transient. Our results confirm the importance of suppressor cells in lepromatous leprosy, and suggest that imbalance between helper and suppressor cells may play a role in the pathogenesis of ENL reactions.
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In a retrospective study of 95 patients seen between 1950 and 1978, the outcome of malignant melanoma levels III, IV and V of Clark's classification was reviewed. Patients with lentigo malignant melanoma were excluded. The overall survival rate was 63% at 5 years and 48% at 10 years. For the whole population of patients significant differences in survival rate were associated with level (p = 0,00002), thickness (less than 2 mm or greater than or equal to 2 mm; p less than 0.0,0001) and histological type (p = 0,02). The significance of prognostic variables taken separately was calculated by the Breslow method and was found to be: p = 0,0005 for thickness, p = 0,0009 for patient's age and p = 0,02 for histological type. In analysis with two variables, including level, thickness was the variable that added most to the information on prognosis supplied by level. For the population of patients with melanoma levels III and IV significant differences in survival rate were associated with thickness (less than 2 mm or greater than or equal to 2 mm; p less than 0,001), though not with level. When the variables were studied separately, thickness and patient's age were significant (p = 0,02 and p = 0,03 respectively). Analysis with two variables, including thickness, showed that age was the variable that added most to the information on prognosis supplied by thickness.
The authors report on a 76-year-old woman presenting with malignant thymoma associated with myasthenia, erythroblastopenia, myositis and giant cell myocarditis. Blood examination showed antinuclear antibodies, anti-platelet antibodies and LE cells. Only one similar case and 22 cases of thymoma associated with giant cell myocarditis have been published. It seems probable that thymomas plays a part in the pathogenesis of giant cell myocarditis.
The clinically normal skin of the lower back of 30 patients with diabetes mellitus was examined, using the direct immunofluorescence technique. No deposit of immunoglobulins or complement (C3) could be demonstrated, while other authors have previously reported lupus-like deposits in diabetes mellitus. As other discrepant studies of skin immunofluorescence have been published, it is suggested that the standards of the various immunopathology laboratories are different. This may explain why the actual value of the lupus band test remains controversial.
T cell subsets and T cell functions were explored in 31 leprosy patients with the following methods: determination of the percentages of the different T cell subpopulations defined by monoclonal antibodies directed at total T cells, helper T cells and suppressor/cytotoxic T cells; measurement of the in vitro proliferative responses to mitogens; study of the concanavalin A-induced suppressive activity, assessed on MLC; measurement of delayed-type hypersensitivity by skin testing. The confrontation between immunological lepromatous patients without type-2 reaction (erythema nodosum leprosum), (2) lepromatous patients without ENL (erythema nodosum leprosum), (2) lepromatous patients was recent ENL and (3) tuberculoid patients. Unexpectedly, groups 1 and 3, although differing strongly in their clinical status and their sensitivity to lepromin (absent in group 1 and strong in group 3), showed a similar immunological profile with a normal percentage of T cells and a normal distribution of T cells among the major T cell subset contrasting with a moderate decrease of proliferative responses to mitogens and impaired delayed-type hypersensitivity reactions. Concanavalin A-induced suppressive activity was type-2 reaction) strongly differed from both other groups, showing striking abnormalities other groups, showing striking abnormalities of the repartition of the T cell subsets, with increased percentages of helper T cells and decreased percentages of suppressor T cells, and elevated proliferative responses to mitogens. Concanavalin A-induced suppressive activity was reduced in most patients of this group. It is suggested that this imbalance between T cell subsets contributes to the occurrence of ENL reactions in lepromatous patients.
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The lupus band test is usually considered as a valuable aid for the diagnosis of lupus erythematous. Involved skin shows positive results in about 900 p. 100 of cases of discoid LE and systemic LE. However, the specificity of this test has been challenged by reports of positive immunofluorescent findings in other cutaneous diseases, like rosacea, in non-cutaneous diseases, and in healthy individuals. Discrepant data from the literature prompted us to report our own experience : direct immunofluorescent examination of skin biopsies was performed in 550 patients from a dermatologic department. Concerning diseased skin, a dermal-epidermal band was observed only in 18 cases of lupus erythematosus, in 4 cases of lepromatous leprosy, in one case of vasculitis, one case of prurigo, and one case of dermatomyositis. Concerning healthy skin, a band was seen only in 8 cases of systemic lupus erythematosus. These results confirm other reports establishing the specificity of the lupus band test.
The plasma of leprosy patients contains high levels of mucoproteins which are deficient in sialic acid. However, due to the increased mucoprotein level, the total sialic acid content of leprous plasma, calculated on protein, is increased when compared with normal human plasma. The low serotonin uptake observed with isolated platelets is probably due to their low sialic acid content. The inability of normal human plasma to correct the diminished serotonin uptake by isolated leprous platelets is in favor of a definite structural change in leprous platelets, related to their low sialic acid content. In patients with active disease and in those with lepra reactions, leprous plasma itself can correct the diminished uptake of serotonin by the isolated platelets. In patients with subsided lepra reactions, the leprous plasma is much less effective. In severe cases, where serotonin uptake is decreased even in platelet rich plasma, desoxyfructo-serotonin increased the uptake of serotonin.
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