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Biomedical subjects

C Blanchet-Bardon

Publications and source records attributed to C Blanchet-Bardon.

At least 109 records · Page 6Linked to original sources
PubMed1979

[Placental sulfatase deficiency and sex-linked recessive ichthyosis. Two cases found in two sisters (author's transl)].

The diagnosis of placental sulfatase deficiency was made at the same time in two sisters who were pregnant. This is the first case history reported of two women who were carriers of this abnormality and who were linked by parentage. The inborn error of metabolism was able to be found in its post-natal state in two of the sons of one of the women in the form of retention cutaneous ichthyosis of a sex-linked type.

Adult↗
PubMed1975

[Correlation between scleroderma and Sjogren's syndrome. Value of systematic biopsy of the labial salivary glands].

Thirty one patients with generalised scleroderma underwent labial biopsy. Sixteen showed a nodular infiltration of the accessory salivary glands indicative of Sjögren's syndrome. In 21, intralobular fibrosis was present. More common in those forms with extensive cutaneous involvement, collagenous fibrosis would appear to be a direct manifestation of scleroderma. The frequency of these abnormalities poses the problem of immunological competence with respect to lymphocytic labial infiltrates in generalised scleroderma.

Biopsy↗
PubMed1975

[Arrector muscle in patients with scleroderma. Histological and histo-enzymological study].

In a previous paper, we have found, at post-mortem examination of three cases of scleroderma, the oesophageal smooth muscle alterations already predicted by physiologists and reported by Treacy. These lesions mostly consist of a loss of the staining properties of the differenciated cytoplasm of the muscle cell, preceding any appearance of interstitial fibrosis. We have already reported these muscle lesions at all other levels of the digestive tract (stomach, small and large bowels), and also in the bladder muscle. In the present study, we have attempted to precise these notions in biopsies and have studied the arrector pili muscles in normal skin of 13 sclerodermic patients by means of histochemical (Regaud, Mallory, etc. ...) and enzyme stains. In all cases, we have been able to identify morphological alterations of the muscle fiber, mostly consisting in an irregular pattern of enzyme properties, especially when diaphorases, dehydrogenases and ATP ases were concerned. This work seems to confirm that the primary site of the sclerodermal lesions is situated in the smooth muscle fiber.

Adenosine Triphosphatases↗
PubMedDate not supplied

[Review of ophthalmologic complications in hereditary bullous epidermolysis. Apropos of 40 cases].

INTRODUCTION: We assessed the frequency of ophthalmologic signs in inherited epidermolysis bullosa and evaluated follow-up. PATIENTS AND METHODS: Forty patients were studied retrospectively. Of the 40 patients, 38 had dystrophic and 2 had junctional epidermolysis bullosa. A complete ocular examination was performed in all cases and repeated if necessary (8 times). RESULTS: Ocular complications were found in 75% of the patients. Corneal anomalies associated or not with refractive anomalies were present. The best corrected visual acuity was less than 3/10 in 20% of the cases. Only 15% had lid modifications. DISCUSSION: In our series, 75% of the cases had ocular anomalies, similar to other series in the literature. Corneal problems dominated. CONCLUSION: Ocular lesions in inherited epidermolysis bullosa are often found and require initial ocular examination. Corneal lesions also need to be followed with optical correction to optimize functional prognosis.

Adolescent↗
PubMedDate not supplied

[Lichen pemphigoid associated with developing hepatitis B in a child].

INTRODUCTION: Lichen planus pemphigoides is a rare acquired auto-immune bullous dermatosis which usually affects adults. Only four cases have been reported in children. We describe a new case of lichen planus pemphigoides in a child unusual by its association with an evolutive hepatitis B and by the occurrence of a lichen planus relapse. CASE REPORT: A 10-year-old African boy has been seen for a pruritic dermatosis with papular lichenoid lesions on the trunk and the limbs and blisters on the lower limbs, both arise on lichen planus lesions and normal skin. The diagnosis of lichen planus pemphigoides was confirmed by histology which showed the features of lichen planus on a papule and of a sub-epidermal split on a bulla and by direct and indirect immunofluorescent studies which revealed an IgG and C3 linear deposit at the dermo-epidermal junction and the presence of circulating IgG anti-basement membrane zone antibodies. Laboratory investigations showed an evolutive hepatitis B (HBsAg +). Healing was obtained by dapsone and topical steroid therapy. Eight months after withdrawal of treatment the patient presented a non-bullous relapse of lichen planus. The histology showed a typical aspect of lichen planus and the immunofluorescence studies were negative. The hepatitis B serology was unchanged. The lesions rapidly improved with topical steroid and coaltar. One year later the patient exhibited few slight lichen planus lesions on the limbs and the hepatitis B serology showed the onset of sero-conversion. DISCUSSION: Lichen planus and lichen planus pemphigoides are probably variants of the same disease. Their successive occurrence in our case report favours this hypothesis as does the association with an hepatitis B.

Administration, Cutaneous↗
PubMedDate not supplied

[Digestive symptoms of scleroderma (author's transl)].

The authors successively study the oral, peri-oral, esophageal, intestinal and anal manifestations of systemic scleroderma. They point out that dental, salivary, and esophageal involvement is of great interest for diagnosis and prognosis. They particularly describe the malabsorption syndrome including anatomical and ultrastructural lesions of the smooth muscle fibers. They present an original study of arrector pili in clinically and histologically non-involved skin of patients undergoing scleroderma. Finally they state that any suspected or diagnosed scleroderma needs a digestive check-up as follows: radiologic examination of the teeth, biopsy of the minor salivary glands of the lower lip, radiologic study of esophageal motility and radioactive C 14 glycocholate test.

Esophageal Diseases↗