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Biomedical subjects

J Maleville

Publications and source records attributed to J Maleville.

At least 73 records · Page 4Linked to original sources
PubMed1988

Generalized epidermolysis bullosa with congenital synechiae, associated malformations and unusual ultrastructure: a new entity?

A male newborn presented at birth with marked skin fragility and widespread erosions of the face, scalp, trunk and extremities. Abnormal associated findings included: cleft palate, hypoplastic low-set ears, bilateral synechiae of the first and second toes, incomplete fusion of both eyelids, and dystrophic nails. There was no evidence of consanguineous parentage. The karyotype was normal. The baby died at 3 days of age because of pulmonary distress. As demonstrated by light and electron microscopy, there were both epidermolytic and dermolytic features. Desmosomes and tonofilaments were decreased in most basal cells. Hemidesmosomes were normal. Anchoring fibrils were rare. This type of epidermolysis bullosa does not fit any known categories of disease.

Abnormalities, Multiple↗
PubMed1988

[The oak processionary caterpillar (Thaumetopoea processiona L.) and man: urticating apparatus and mechanism of action].

The urticating apparatus of the oak processionary caterpillar was studied by electron microscopy at times when this species was exceptionally abundant in France. Proteins of urticating hairs were studied by electrophoresis on acrylamide gel and were compared with thaumetopoein, an urticating protein of the pine processionary caterpillar, using immunological techniques. A thaumetopoein-like protein was found to be present in oak processionary urticating hairs. This protein has a histamine-releasing effect in the guinea-pig. Contact dermatitis caused by the oak processionary caterpillar is described and compared to that produced by the pine processionary caterpillar.

Animals↗
PubMed1988

[Immunologic changes in linear scleroderma in children. Apropos of 11 cases].

Immunologic data collected in 11 children (6 girls and 3 boys under fourteen) presenting with linear scleroderma were analysed in a retrospective study: 2 children presented with superficial linear scleroderma, 6 with monomelic scleroderma, 1 with dimelic scleroderma, 1 with the "en coup de sabre" variety associated with dimelic homolateral involvement, and another with "en coup de sabre" scleroderma combined with facial hemiatrophy: Antinuclear antibodies (ANA) were demonstrated in 9/11 cases (i. e. 81 p. 100). The immunofluorescence staining pattern was homogeneous in all nine with a low titer (less than 250 in 5 of them). ANA to single stranded DNA was present in 1/3. The demonstration of ANA in these 9 children was correlated with deep or extensive sclerosis with muscular involvement in 7. But neither the presence nor the titer of ANA were correlated with the subsequent development of osteoarticular sequelae. The level of total complement appeared to be lowered in 3/8 cases. No renal involvement was demonstrated. Blood tests for circulating immune complexes were positive in 4/8 patients. Skin biopsy for direct immunofluorescence was performed in 6 children and demonstrated immunoglobulin deposits in 4: three had IgM fixation on the dermo-epidermal junction, and one had speckled fixation of IgG on epidermal nuclei (this has not previously been reported in localized scleroderma). There data highlight: a--the high frequency of ANA in linear scleroderma.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

Postgranulomatous anetoderma associated with Takayasu's arteritis in a child.

Takayasu's arteritis (TA) is a rare chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum and subcutaneous inflammatory lesions of the leg are the most frequent. We studied a boy with TA in whom a papular rash of the trunk preceded the onset of vascular symptoms by many years. Histologically, the lesions were superficial and consisted of middermal noncaseating tuberculoid granulomas, which progressed to atrophy and anetoderma because of elastic network disruption. Granulomas were also found in synovial tissue but not in a temporal artery biopsy specimen, which showed only intimal hyperplasia. Our observations suggest that vascular and skin lesions with elastic tissue may both result from a common granulomatous hypersensitivity process.

Aortic Arch Syndromes↗
PubMed1987

Collagen studies in congenital cutis laxa.

A case of congenital cutis laxa (CCL) of unclear mode of inheritance associated with multiple pulmonary artery branch stenosis was extensively investigated to assess possible correlations between clinical, ultrastructural, and biochemical features. Light microscopy revealed that elastic fibers were absent in the papillary dermis, while hypoplastic elsewhere. Transmission electron microscopy showed a poor elastin matrix content in some elastic fibers, variable diameters of collagen fibrils, and abundant glycogen granules in most dermal cells. Measurement of collagen fibril diameters, using an image analyzer, was carried out in the patient and two age- and site-matched controls. A bimodal distribution was found in the upper reticular dermis of the patient. In vitro analysis of collagen in skin fibroblast cultures of the patient showed increased collagen synthesis with a balanced production of type I and type III procollagens. Our study confirms that CCL represents a disorder both of collagen and elastic connective tissue.

Cells, Cultured↗
PubMed1987

Clinical epidemiology of symptomatic primary herpetic infection in children. A study of 50 cases.

We have studied a series of 50 children with clinical primary herpetic infection during 1975-1985. Our data confirm: absence of sex differences and seasonal variations, a peak age of incidence between 6 and 24 months, a prevalence of patients of lower social status, evidence of recurrent herpes labialis as the most frequent source of infection, and frequency of herpes simplex virus 1 gingivostomatitis. The relevant findings of this study were as follows: herpes simplex virus 2 was isolated in 10% of the patients, 6% of cases occurred in the first six months of life, infection was multifocal in 36% of cases, autoinoculation was a frequent route of transmission of genital primary infection in young children, herpetic Kaposi-Juliusberg's pustulosis in infants with atopic dermatitis was the most severe presentation of primary infection and should be more adequately prevented.

Child↗
PubMed1986

[3 cases of neonatal cyto-steatonecrosis. Physiopathological discussion].

Three cases of subcutaneous fat necrosis of the newborn (SFNN) are presented. They occurred after a therapeutic coma induced by intravenous barbiturates to control convulsions unresponsive to conventional treatment. Two patients had widespread lesions. Histopathology showed prominent vascular involvement. Since 1970, 26 cases of SFNN have been published. Among the aetiological factors already described, hypothermia and ischaemia play a major role. It is suggested that brown adipose tissue might be of importance in the pathophysiology of subdural fat necrosis of the newborn.

Adipose Tissue, Brown↗
PubMed1986

Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child.

A 7-year-old girl developed recurrent febrile nodules and subcutaneous plaques on her arm, shoulder, and face. Primary acquired toxoplasmosis was diagnosed at onset, because of associated lymphadenopathy and detection of specific IgM. Skin lesions were responsive to therapy with systemic steroids but progressed to atrophy and poikiloderma within a few months. Subsequently, chloroquine therapy has been instituted and no relapse has occurred. Histopathologic findings and direct immunofluorescence studies were diagnostic of lupus erythematosus panniculitis. Electron microscopy showed lamellar inclusions within mast cells. Results of laboratory investigations disclosed leukopenia and hypocomplementemia with low C2 serum levels. A family study of the complement system, including C4 and Bf phenotyping and HLA A, B, and DR haplotyping, revealed the carriage of both C2 and C4A null alleles in the propositus. This observation suggests an additional link between lupus erythematosus panniculitis and the remainder of the lupus erythematosus clinical spectrum.

Adolescent↗
PubMed1986

Solitary Langerhans cell histiocytoma.

Biopsy of a solitary tumor of the buttock in a 3-month-old girl was diagnosed as a histiocytic proliferation suggestive of histiocytosis X. Electron microscopy showed Birbeck granules and dense myelinlike bodies within the cytoplasm of the tumor cells. An immunoperoxidase study, using a panel of monoclonal antibodies (OKT6, OKT4, and OKM1) and a polyclonal anti-S100 protein antibody, showed positive staining for OKT6 and OM1 and moderately positive staining for OKT4 and S100. After surgery to remove the tumor, visceral involvement could not be demonstrated during a 20-month follow-up.

Buttocks↗
PubMed1986

Thaumetopoein: an urticating protein from the hairs and integument of the pine processionary caterpillar (Thaumetopoea pityocampa Schiff., Lepidoptera, Thaumetopoeidae).

Hairs of the Thaumetopoea pityocampa caterpillar (Lepidoptera) cause a cutaneous reaction in man and animals. The irritating fraction extracted from hairs contains soluble proteins which were separated by various electrophoretic and immunoelectrophoretic techniques. Some of these proteins are present also in cuticle and haemolymph. One protein of 28,000 mol. wt is hair specific and caused a reaction in pig skin identical to that produced by hair extract. It is therefore an urticating protein which we have named thaumetopoein. This protein is formed of two subunits of molecular weights 13,000 and 15,000. It is present in large quantities in the glands producing urticating hairs.

Animals↗
PubMed1986

Gianotti-Crosti syndrome: a study of 26 cases.

We have studied 26 patients presenting with a symmetrical papular or papulovesicular acrolocated eruption of more than 10 days duration. Mean age at onset was 2 years (range 10 months to 5.75 years). Lymphadenopathy was noted in eight cases, and hepatomegaly in one case. In 12 cases, histopathology and direct immunofluorescence were non-contributory. Cytolytic hepatitis occurred in one case and was associated with HBs antigenemia. A history of recent immunization was given in two cases. There was serological evidence of recent Epstein-Barr virus infection in seven out of 13 cases tested. Coxsackie B viruses were isolated from three patients, and cytomegalovirus was probably associated with the syndrome in one case. We conclude that the Gianotti-Crosti syndrome is not rare in France, and that non-hepatitis B virus (HBV)-associated cases are more frequent than the classical HBV-associated papular acrodermatitis of childhood.

Acrodermatitis↗