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Biomedical subjects

F Cambazard

Publications and source records attributed to F Cambazard.

At least 109 records · Page 6Linked to original sources
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

[Involvement of the urinary tract in a syndrome of congenital epidermolysis bullosa and atresia of the pylorus].

Epidermolysis bullosa associated with pyloric atresia is a rare autosomal recessive condition, usually fatal in the first few months of life. Since 1983 urinary tract disease is known to be another manifestation of this syndrome which becomes the main problem in children surviving beyond infancy. The case of a 2 1/2 year-old boy with mild cutaneous manifestations of junctional epidermolysis bullosa, corrected pyloric atresia and renal disease is reported. Hematuria, dysuria, obstruction of the uretero-vesical junction and worsening hydronephrosis led to bilateral ureterostomy (ureteral reimplantation was not attempted because the thickness of the bladder wall and the extensive ureteral fibrosis).

Child, Preschool↗
PubMed1986

Lipoid proteinosis with pseudomembranous conjunctivitis.

Lipoid proteinosis is a rare autosomal recessive condition characterized by a diffuse mucocutaneous infiltration with histopathologic deposits that are positive for periodic acid-Schiff reagent and a sudanophil substance. We present a 9-year-old boy with lipoid proteinosis. His parents are siblings. He had classical manifestations, such as statural-ponderal delay, hoarseness, yellowish skin papules, atrophic scars, and moniliform blepharosis. A transmission deafness was also found. There were none of the intracranial calcifications that are usual at this age. A pseudomembranous conjunctivitis was surgically treated when he was 4 months old. This clinical manifestation has not hitherto been described in lipoid proteinosis.

Child↗
PubMed1986

Skin and mucosal manifestations in vitamin deficiency.

The skin and mucosal changes in vitamin deficiency are described. Pellagra, which is the oldest known cutaneous manifestation among vitamin deficiencies, is reviewed. Cutaneous alterations caused by deficiency of the water-soluble vitamins B6, C, B1 and biotin, B12, folic acid, and riboflavin result in more mucosal alterations and are discussed. Alterations caused by fat-soluble vitamin deficiencies (vitamins A and K) are also considered.

Ascorbic Acid Deficiency↗
PubMed1986

[Cyclosporin in the treatment of bullous pemphigoid: preliminary study].

Bullous pemphigoid is a typical autoimmune disease. It is classically treated with systemic corticosteroids alone or combined with immunosuppressants. Cyclosporine A (CyA), a new immunosuppressive agent with high activity in organs and bone marrow transplantation, could be expected to prove effective in this disease owing to its action on lymphocytes. Seven patients with bullous pemphigoid were treated with CyA in daily doses of 6 to 8 mg/kg bodyweight. Treatment was monitored by measurements of serum CyA, creatinine and liver enzyme levels. The effectiveness of treatment was assessed on clinical changes. The seven cases are dealt with individually, with a brief case-report for each of them. The only side-effects observed were reversible rises in serum creatinine levels and hypertrichosis in two cases; these are usual reactions to the drug. Hormonal assays were normal in two female patients. Concerning results, our patients fell into two groups. Among those treated with CyA alone there were two failures and two sustained satisfactory results. Treatment was successful in all patients treated with CyA during relapses under corticosteroid therapy, but two patients relapsed after CyA was discontinued. It is concluded that CyA is of no interest in the acute phase of bullous pemphigoid, that the long-term stability of the results obtained is doubtful and that this potentially nephrotoxic drug should be avoided or administered with extreme caution in elderly people, since their renal function may be at the limit of normality.

Adrenal Cortex Hormones↗
PubMed1986

[Ito's hypomelanosis. Review of the literature apropos of 3 cases].

Initially described as incontinentia pigmenti achromians, Ito's hypomelanosis is a congenital disease characterized dermatologically by depigmented maculae arranged in a specific pattern. These maculae appear suddenly, unheralded by an inflammatory process, and are arranged on the limbs as lines and on the trunk as whirlwinds or mottled cakes. Other abnormalities, notably neurological, ophthalmic or musculoskeletal may be associated with this spray-like depigmentation of the skin. We report here three cases of this disease, which is probably more common than the scarcity of cases hitherto published would suggest. Case no. 1. A 9-year-old boy presented since the age of 5 with generalized convulsive fits predominant on the right side. Neurological examinations between fits were negative. IQ, FO and CT scans of the brain were normal. During the first months of life, the child had developed a spray-like depigmentation on the right half of his back and on the anterior and posterior aspects of his right arm. Case no. 2. A 2-year-old girl was examined for a cutaneous depigmentation which had developed when she was about 3 months' old and had progressively extended from her left knee to her left hypochondrium. Case no. 3. An 8-year-old girl presented with mental retardation and myopia, but also with a spray-like depigmentation on the left part of her chest. The skin lesion had been noticed by her parents after she had exposed herself to the sun in a tropical country. Histological examination performed in the first two patients showed some degree of hypopigmentation of the epidermis without pigmentary incontinence.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1986

[Cutaneous manifestations of periodic disease].

The authors report the case of an 8 year-old girl admitted for an erysipelas-like rash of the lower limbs and an episode of Henoch-Schönlein purpura. These manifestations were subsequently attributed to familial mediterranean fever. This case report illustrates the polymorphism of cutaneous manifestations in this disease.

Child↗
PubMed1985

[Mycobacterium chelonei cutaneous infections. General review apropos of a case].

Mycobacterium chelonei is a facultative pathogen which exists as a saprophyte in the environment and rarely produces clinical manifestations in humans. We describe a 62-year-old woman, long-term treated with low-dose steroids for severe asthma, who presented sporotrichoid-like lesions on one leg. These lesions appeared two months after a cat-scratch and were present for six months. Histological examination showed acute inflammation with polymorphonuclear infiltration without tuberculoid granuloma nor caseation necrosis. Ziehl-Neelsen stains were negative. Mycobacteria were found by direct examination and Mycobacterium chelonei chelonei was identified by culture. Treatment with isoniazid, rifampicin and ethambutol was given for one month and followed by complete resolution of the lesions, though each of these antibiotics was not effective in vitro. The literature about Mycobacterium chelonei infections is reviewed and the role of the antibiotherapy in clinical recovery is discussed.

Asthma↗
PubMed1984

[Pseudoscleroderma and sclerodermiform states].

Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism. Linear pseudo-scleroderma is suggested by the following scleroderma-like conditions: facial hemiatrophy, acrodermatitis atrophicans, melorheostosis, pseudo-scleroderma after corticosteroid injection, and cutaneous lesions in carcinoid syndrome. Scleroderma in plaque must be differentiated from hypodermitis sclerotisans, panatrophy and localized lipoatrophies, hypodermitis after vitamin K injection, basal cell carcinoma, necrobiosis lipoidica, vitiligo, chronic radiodermatitis, cutaneous lymphatic invasion. Scleroderma-like changes after drug injection (vitamin B12, progestin), anetoderma barely resemble morphea guttata.

Adipose Tissue↗
PubMed1983

T cell subsets and Langerhans cells in skin tumours.

The purpose of this study was to examine the phenotype of the cutaneous immunocompetent cells and Langerhans cells in malignant and benign tumour infiltrates (basal cell and squamous cell carcinomas, malignant melanoma, seborrheic keratosis and naevus) by the use of monoclonal antibodies directed against T cell populations and Langerhans cells. This in situ investigation indicates that the lymphocytes participating in the inflammatory reaction around skin tumours are mainly of the cytotoxic/suppressor class. It suggests, moreover, that interaction occurs in the skin between T lymphocytes and HLA-DR suppressing cells. The in situ study of the inflammatory immune response should be a useful complement to in vitro investigations in the exploration of immune reaction against tumours of the skin.

Basal Cell Carcinoma↗