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Biomedical subjects

G Guillet

Publications and source records attributed to G Guillet.

At least 127 records · Page 7Linked to original sources
PubMed1988

Anti-Ro-positive lupus and hereditary angioneurotic edema. A 7-year follow-up with worsening of lupus under danazol treatment.

A 24-year-old woman, having familial hereditary angioneurotic edema (HAE) treated with danazol, developed an anti-Ro-positive lupus featured by a mild erythematous eruption that seemed initially to decrease and then worsened under danazol, and dramatically improved after danazol was withdrawn. The relationship between lupus and HAE is discussed with special reference to the hormonal sensitivity of autoimmune diseases. It is possible that the responsibility of danazol as well as the immunogenetic background can explain the clinical course of this association.

Adult↗
PubMed1988

[Systemic scleroderma in children].

A new case of systemic scleroderma in a child is reported. The frequency of this disease is much lower than focal scleroderma and than other connective diseases in childhood. Kidney and heart involvement is much less common than in adults. Raynaud's phenomenon and scleroderma however, as in this case, are often severe. The appropriate treatment has to be efficient towards sclerosis (D-penicillamine, corticosteroids), Raynaud's phenomenon (protection from cold, nifedipine) and other manifestations (prevention of gastric ulceration, physical therapy) and should consider every possible adverse effect on a growing organism.

Adrenal Cortex Hormones↗
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

Linear cutaneous neuromas (dermatoneurie en stries): a limited phakomatosis with striated pigmentation corresponding to cutaneous hyperneury (featuring multiple endocrine neoplasia syndrome?).

A 60-year-old woman developed a progressive linear pigmentation on the trunk. Skin biopsy demonstrated an increase in the number and size of neural fibers in the dermis. Clinical and paraclinical investigations of this unusual disease showed findings similar to the hereditary type of Sipple syndrome (multiple endocrine neoplasia syndrome, Type 2b). Such findings as Marfanoid habitus, abnormal electromyography and hypertrophy of the corneal nerves suggest that our patient's disease and Sipple syndrome are identical. However, such other symptoms of Sipple syndrome as presence of endocrine tumors are absent. Questions of long-term prognosis and physiopathogenicity are raised, since no nerve growth factor was detected in this patient. The name of linear cutaneous neuromas is suggested for the patient's lesions, and a possible association with the pigmentation and cutaneous neuromas of the MEN 2b syndrome.

Biopsy↗
PubMed1987

Keratinocyte T6 antigen expression after PUVA therapy.

We report T6 antigen expression on keratinocytes in 11 cutaneous T lymphomas treated by PUVA therapy. This staining was absent before treatment. T6 reactivity was strictly limited to cell membrane. The nature of this expression is discussed, and it is suggested that it could be attributed to a passive diffusion from Langerhans's cells.

Antibodies, Monoclonal↗
PubMed1987

[Keratosis follicularis decalvans: nosological discussion of Siemens' disease. Apropos of 3 cases].

We describe 3 cases of keratosis follicularis decalvans (Siemens' disease): a 15 year old boy and a 7 year old boy and his father. They represent 2 different patterns of the disease with different clinical courses and genetic background: an autosomal dominant type of good prognosis with elevated argininemia and absence of follicular atrophy in both father and son, a sporadic type, clinically severe, with follicular atrophy. This raises the question of the nosology of the so-called Siemens' disease, since it includes actually different diseases of variable prognosis.

Adolescent↗
PubMed1987

[Juvenile digital fibroma: apropos of 2 cases].

The authors report 2 cases of infantile digital fibromatosis. These benign tumours occur in newborns and infants, involving mainly the extremities (fingers, toes). Its prevalence is probably higher than the reported cases. The pathogenesis and treatment are discussed. The diagnosis of such fibrous tumours is useful since surgical treatment can be avoided in a majority of cases.

Female↗
PubMed1987

[Pyoderma gangrenosum and pelvispondylitis without patent intestinal disease. Apropos of a case].

The authors report a case of pyoderma gangrenosum associated with a positive B27 pelvispondylitis. The search for a hemorrhagic rectocolitis, possible link between the two syndromes, revealed to be negative. Pelvispondylitis is an unusual complication of pyoderma gangrenosum. Review of the literature shows only one case of sacroiliitis, beside seronegative polyarthritis unrelated to HLA B27.

Adult↗