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Biomedical subjects

Y Privat

Publications and source records attributed to Y Privat.

At least 37 records · Page 2Linked to original sources
PubMed1989

[Value of colchicine in treating acquired epidermolysis bullosa].

In July 1986, a 46-year old male patient was admitted for a bullous skin disease of 4 years' duration. The disease fulfilled all the criteria of epidermolysis bullosa acquisita (EBA), as laid down by Roenigk and Pearson. Despite a guided investigation, none of the diseases classically associated with EBA could be found, but it must be noted that immunological stigmata of an old hepatitis B were present. After failures or partial results with prednisone alone (1 mg/kg/day from August to October 1986), then methotrexate (30 mg/week) combined with prednisone (20 mg/day), the patient was treated with colchicine in doses of 2 mg per day, and within a fortnight a dramatic improvement of buccal mucosal lesions and cutaneous fragility was observed. Colchicine was withdrawn, but 5 days later bullae and erosions of the mucosa reappeared. The reintroduction of colchicine in the same doses (2 mg/day) resulted in remission of the lesions. The disease has now remained stable for one year under colchicine 1 mg/day. Four attempts at reducing this dosage to 1 mg every other day brought about the recurrence of a few bullae and of cutaneous fragility. To our knowledge, colchicine has not yet been reported to be effective in the treatment of EBA. Its effectiveness may be due, to a great extent, to its immunomodulating properties, notably on some functions of neutrophils the role of which in the pathogenesis of bullous lesions seems to have been established. By modulating collagen synthesis and collagenase activity colchicine might induce structural modifications of the EBA antigen, identified as the carboxyterminal group of type VII collagen, and inhibit its recognition by autoantibodies.

Colchicine↗
PubMed1988

Residual plasma concentrations of acitretin (Ro 10-1670) and its metabolite (Ro 13-7652) after chronic administration.

In order to study the elimination of Ro 10-1670 (acitretin) and Ro 13-7652, its 13-cis-isomeric metabolite after chronic administration, determination of the residual plasma concentrations of these two compounds were assayed in 9 patients in a range of 22-30 days following cessation of a 2- to 7.5-month course with daily oral doses of 10-50 mg of acitretin. A highly sensitive HPLC method was used (2 ng/ml). Residual plasma concentrations of Ro 10-1670 were below the quantification limit in all cases. Residual plasma concentrations of Ro 13-7652 were below the quantification limit in 7 cases and respectively of 3 and 4 ng/ml in the other 2 cases. These data appear to confirm the absence of storage of acitretin even after an extended course of therapy.

Acitretin↗
PubMed1988

Multiple clustered dermatofibromas. Evolution over 20 years.

A 37 year-old man developed, over 20 years, multiple clustered dermatofibromas on the left thigh. Three similar cases have been described previously and the benign course of this lesion has been referred to. Analysis of collagen amino acids revealed a sharp increase of hydroxylysine on the fibrous central zone, suggesting an abnormal accumulation of type IV collagen.

Adult↗
PubMed1987

Collagen biosynthesis and isomorphism in a case of Ehlers-Danlos syndrome type VI.

Collagen metabolism was studied in fibroblasts grown from a skin biopsy specimen of a patient who presented the striking clinical features of Ehlers-Danlos syndrome and, in particular, hyperextensibility of the skin, hypermobility of the joints, and kyphoscoliosis. A reduction in lysine hydroxylation, characteristic of Ehlers-Danlos Type VI, was observed after labelling of the collagen with 14C-proline and 3H-lysine. Other modifications in the collagen metabolism of fibroblast cultures were noted, including an increase in collagen and total protein synthesis, and an increase in both the Type I and Type III collagen. The percentage of Type III collagen was, however, lower than in the control fibroblasts. The results point out the complexity of collagen disturbances in Ehlers-Danlos Type VI.

Adult↗
PubMed1987

Severe erosive lichen planus and polycythemia vera in an adolescent.

Erosive lichen planus developed in a 12-year-old adolescent also presenting polycythemia vera (Vaquez' disease). Though a casual association cannot be excluded, the rarity of the two diseases in children suggests a pathogenetic link, possibly involving an abnormal hematologic stem cell line.

Biopsy↗
PubMed1987

[Metastatic Darier-Ferrand dermatofibrosarcoma. Review of the literature apropos of a case].

We report the case of a 56-year old male patient with pulmonary, abdominal and pelvic metastases from a dermatofibrosarcoma (DFS) which had developed 23 years previously in the right scapulo-humeral region and had recurred twice after the first excision, in 1983. Surgery was only performed once. The most remarkable feature of the disease was the progressive transformation, with each recurrence, of a typical DFS into a poorly differentiated and highly malignant tumour, about as active as a fibrosarcoma. A review of the literature yielded 31 cases of histologically proven metastatic DFS. Although the actual incidence of metastases is difficult to determine with accuracy, their frequency may be estimated at 3 p. 100 (most probably a maximum figure). The disease spreads mostly through the blood, but also sometimes through the blood and lymph. However, since lymphatic involvement is rare the usefulness of lymph node excision is extremely doubtful. Pulmonary lesions are the most frequent ones, but various other organs may be affected, although this seldom applies to the liver. Following a first excision, metastases occur within 6 years on average (range: 1 to 33 years). This mean delay is superior to the mean follow-up period for most of the important series without metastases. The histopathological picture is usually one of typical DFS but in some cases, including ours, it is much less typical and may even be frankly malignant. A few reports mention the presence of histiocytic infiltrates associated with the fibroblasts. The prognosis in patients with metastases is particularly poor: in most of the cases reported the patient died within the year following the discovery of metastatic lesions. The very high progressivity of the secondary lesions contrasts with the slow course of the primary tumour. None of the treatments tried (surgery, radiotherapy, chemotherapy) has resulted in a significant prolongation of life. No correlation can be established between the occurrence of metastases and the following parameters taken together: patient's age and sex, tim elapsed between the appearance of the tumour and the date of exicision, and histological features of the initial tumour. The development and number of recurrences seems to be the most significant predictive factor as regards the risk of metastasis. The noxious role of incomplete and/or repeated surgery creating vascular breaks, disrupting the defense barriers and encouraging malignancy in some cases has been mentioned by most authors; it emphasizes the value of the widest possible initial surgery.(ABSTRACT TRUNCATED AT 400 WORDS)

Fibrosarcoma↗