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D Barrut

Publications and source records attributed to D Barrut.

At least 19 recordsLinked to original sources

[Chicken pox recurrence revealing a renal adenocarcinoma in an adult].

A new episode of chicken pox in adults who had a well documented infection previously is usually observed in immunocompromised individuals. The principal immunodeficiency factors are hematology diseases, acquired immunodeficiency disease and old age. We report here the case of a young woman who after a contaminating contact presented a recurrence of typical chicken pox. Morphological investigations evidenced a right kidney tumor which pathology revealed to be a renal adenocarcinoma. We discuss this pathological association and review cases reported in the literature.

Acyclovir↗

Excision repair defect in Rothmund Thomson syndrome.

Rothmund Thomson syndrome is a rare autosomal recessive skin disorder. The main clinical feature is poikiloderma appearing in early childhood associated with skeletal abnormalities. Early occurrence of malignancies is another relevant feature. Here we describe the clinical features of 2 patients with Rothmund Thomson syndrome who were investigated for the in vitro DNA repair capacities of blood cells following UVC radiation exposure. DNA excision repair, assessed with unscheduled DNA synthesis following UVC exposure, was decreased in both patients. Such a defect might explain the patients' sensitivity to sunlight and the relatively high risk of cancer associated with this syndrome.

Adolescent↗

[Congenital eccrine angiomatous hamartoma].

BACKGROUND: Eccrine angiomatous hamartoma is an uncommon skin disease with vascular and sudoral components: less than thirty cases have been reported in the literature. CASE REPORT: A 3.5 month-old female infant developed a painful angiomatous plaque on the abdomen which was first seen 15 days after birth. Histology showed numerous vessels and eccrine sudoral glands in the dermis. Exeresis could not be complete and was followed for 15 years. There has been a slight hyperpigmentation with localized hyperhidrosis over the plaque. This hypersudation confirms the diagnosis which could not be confirmed on the initial biopsies. DISCUSSION: This case of congenital eccrine angiomatous hamartoma could only be diagnosed late in the clinical course. This is the first case report on the abdomen, 80 p. 100 of the previous reports were on the limbs. After a 15 year follow-up, there was no progression of the hamartoma and pain regressed gradually. Without secondary development of localized hyperhidrosis, the diagnosis could not have been made. Careful comparison of pathology findings and clinical expression are essential for the diagnosis of eccrine angiomatous hamartoma.

Abdominal Muscles↗

[Disseminated cutaneous neurilemmomatosis].

INTRODUCTION: Neurilemmomatosis is a rare, non-inherited disease which has several features similar to type I neurofibromatosis. CASE REPORT: A 30-year-old man had more than 300 cutaneomucosal tumours. Most were sessile and of firm consistency on normal coloured skin. Histology reported masses of fusiform dermal cells S100 protein (+), NSE (-), actin (-). The cells had a polygonal ultrastructure with fine ramifications. Neurilemmomatosis (schwannomatosis) was diagnosed. No neurological or systemic disorder could be detected. The most bothersome tumors were destroyed. DISCUSSION: This is a typical anatomoclinical presentation of cutaneous neurilemmomatosis. This exceptional state raises the problem of differential diagnosis and nosological distinction from von Recklinhausen's disease. A characteristic feature in this case was the total absence of any malformation or associated tumour, notably neurological tumours. To our knowledge, this is the only documented case of disseminated neurilemmomatosis strictly limited to the skin.

Adult↗

[Acquired atrophic pigmented band-like lesions following Blaschko's lines].

Over a seventeen years period we observed 5 patients presenting with very similar lesions the equivalent of which we could not find in the literature. These were pigmented and more or less atrophic bands which exactly followed Blaschko's lines. The lesions appeared during childhood or adolescence, between the ages of 6 and 20 years and always were unilateral. In all cases they were located on the trunk where they formed a recumbent "S" pattern characteristic of Blaschko's lines. They often started 3 to 6 cm away from the posterior midline and ended precisely on the anterior midline. These bands varied in number and in 3 out of 5 cases they were associated with pigmented atrophic bands or lines on the limbs of the same side. The intensity of pigmentation and atrophy was variable. The lesions were asymptomatic and only caused a cosmetic prejudice. They were perfectly fixed, and during a 2 to 30 years observation period they remained unmodified. The five skin biopsies performed on 3 patients showed no abnormality of the epidermis other than irregular and moderate hyperpigmentation of its deep part. In the dermis, there was no distinct pigment incontinence, no inflammation and no alteration of connective tissue texture; the elastic network was invariably normal. The impression of skin atrophy was not due to true dermal hypoplasia and perhaps corresponded to atrophy of the subcutaneous cellular tissue. No biological disturbance was observed. These lesions can easily be distinguished from epidermic naevi, incontinentia pigmenti and areas of hypoplastic dermis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Surgery of pulmonary metastasis from malignant melanoma. Results and criteria of surgical excision].

Lung metastases from malignant melanoma are frequent and they often inaugurate the metastatic stage. Exceptionally, they present as one or a few nodules, and in the absence of any other secondary lesion these cases raise the problem of surgical eradication. A retrospective multicentre study was carried out in a series of 38 patients and its results were compared to the data obtained from a review of 435 published cases in order to assess the value of surgery in terms of survival and to delimit its indications as closely as possible. Our series of 38 patients comprised 20 men and 18 women aged from 22 to 93 years (mean 51 years, median 55 years). The primary tumour was located in the trunk in 47 p. 100 of the cases; it was nodular in 33 p. 100 and superficial but extensive in 37.5 p. 100. The time elapsed before the metastases appeared varied from 0 to 108 months (median 40 months). Surgery had been radical in 70 p. 100 of the patients and usually limited, tumorectomies and segmentectomies accounting for 51 p. 100 of the operations. RESULTS. In this series the duration of survival varied between 2 and 144 months (mean 26 months, median close to 15 months), with a 20 p. 100 probability of survival at 5 years (fig. 1). Disease free survival varied from 0 to 144 months (mean 22.5 months, median 10.5 months) (fig. 2, curve 1). The parameters of response as regards patients, primary tumour, metastases and treatment were analysed. Response was uninfluenced by sex and slightly influenced by age, with a difference of borderline significance between subjects under and over 50. The primary tumour characteristics did not affect survival, and the features of metastases were of extremely varied importance. The number of operable metastases was not determinant. On the other hand, the presence of mediastinal lesions, either isolated or associated with lung lesions, worsened the prognosis of terms of survival and much more significantly so in terms of remission (fig. 3 and 4). The evaluation of evolutive characteristics, such as date of appearance and tumour doubling time, was inconclusive. Survival was of the same duration after wide and limited surgery, so that tumorectomy or segmentectomy should preferably be performed. The results of surgical treatment were determinant, with a highly significant difference in survival between radical and incomplete surgery (fig. 5 and fig. 2, curve 2). DISCUSSION. The median survival of patients operated upon for lung metastases is diversely evaluated in the literature as 8 to 29 months (table V), the mean figure of 16 months being virtually the same as that of our series. In this, as in most of the previously published series, the maximum duration of survival was beyond 8 to 10 years. The mean survival rate at 5 years is very close to the one we have recorded (20 p. 100) (table V). Compared with other treatments of lung metastases, surgery may be considered as capable of prolonging survival by 6 months; this is not much unless we add the possibility of a 5-year survival in 1 out of 5 operated patients and the possibility of a survival exceeding 8 or 10 years in 2 to 5 p. 100 of the cases. Some prognostic factors seem to constitute positive or negative criteria of operability. This is the case with mediastinal lesions which may consist of a metastasis of metastasis or of a lymph node invasion associated or not with the lung lesion, but in any case correspond to the involvement of more than one site. Mediastinal lesions must be systematically looked for and treated as contraindications of surgery, as shown by the differences in survival recorded in our series. Opinions differ as regards the value of evolutive parameters of the metastasis. For some authors, a more than 5 years interval before the metastasis appears is associated with a good chance of prolonged survival, whereas a less than 6 months or 1 year interval reflects a steadily high progressiveness and in practice precludes surgery. The value of the

Adult↗

[Ichthyosis and steroid sulfatase: study of enzymatic activity in leukocytes and fibroblasts according to the sex and type of ichthyosis].

Steroidsulfatase and arylsulfatase C were determined in fibroblasts and/or leukocytes of patients affected with different types of ichthyosis. Of the 21 patients studied, 11 showed clinical characteristics of X-linked ichthyosis (XLI) and a deficiency of these 2 enzymatic activities. Patients affected with other types of ichthyosis showed no enzymatic deficiency. In XLI families diagnosis of heterozygotes was performed by enzymatic measurements in the 5 patients' mothers studied. In 2 families enzymatic activities were studied in patients' sisters. The validity of these different enzymatic measurements is discussed.

Adolescent↗

[Familial Degos' atrophic papulosis (mother-daughter)].

Degos' disease (malignant atrophic papulosis) occurring in mother and daughter is described. Mother's eruption is composed of multiple characteristic lesions (up to 600). She does not have any gastrointestinal, neurological nor other visceral manifestations after a 9-year course. One year after the onset of the disease, she had a daughter. The pregnancy was normal and the newborn did not have any cutaneous manifestation. The daughter's first skin lesion appeared when she was 3-months old. She has now been under observation for nearly nine years and developed only four skin lesions without any visceral manifestation. Laboratory investigations show an increase in fibrinogen in both mother and child, without any sign of disseminated intravascular coagulation. The research for "slow virus" by a primate's intravascular inoculation of mother's skin lesion in negative.

Adult↗

[Treatment of Jessner-Kanof disease with thalidomide].

Five patients present a Jessner-Kanof's lymphocytic infiltration of the skin with 6-year duration for 4 of them. The skin lesions are numerous on the face and the back and characteristic of this disease. Histologic examination shows a lymphocytic infiltrate in the dermis without any modification of epidermis. Direct immunofluorescent study is negative in all cases. In 3 cases, response to chloroquine had been inconsistent or negative. The 5 cases have a excellent result with 100 mg/day of Thalidomide. In 4 cases the skin lesions reappear when treatment is stopped. With a continuous treatment of 25-50 mg/day for more than 2 years, 3 of 5 patients have normal skin. Thalidomide appears to be the best treatment of Jessner-Kanof's disease.

Adult↗