Search PubMed⌕ Search

Biomedical subjects

J Bazex

Publications and source records attributed to J Bazex.

At least 145 records · Page 8Linked to original sources
PubMed1994

[Follicular atrophoderma, basal cell proliferation and hypotrichosis (Bazex-Dupré-Christol syndrome). A study in 2 families].

We describe a new case of Bazex, Dupré and Christol (BDC) syndrome in a family (family B...) where the disease affects four generations. Moreover, we report the clinical and genealogical evolution of the first family (family A...), described in 1966 by Bazex. A review of the literature has enabled us to collect the various symptoms of BDC syndrome, to discuss the relationship of this syndrome with basal cell naevomatosis and X-linked dominant chondrodysplasia punctata, and to discuss the kind of heredity. In family B..., two patients were examined. A one-month-old girl, with hypotrichosis and pseudo pili torti, and her 29-year-old father, with hypotrichosis and dorsal follicular atrophoderma of the hands. In this family, the paternal grand mother, one of her sisters, and the great grand father were also affected, but no basal cell carcinomas were found. In family A..., five patients were followed up. Four brothers, adult men aged 43 to 55 years initially presented only dorsal follicular atrophoderma the hands and face, hypotrichosis and numerous basal cell carcinomas of the face, especially in palpebral, temporal, nasal and forehead sites. Surgery was required and gave better results than radiotherapy, which left sites of sclerosis. Their mother was also affected and had been treated for numerous basal cell carcinomas of the face. The other members of the same generation (two women, two men) were unaffected. But the uncle and the grand father had the disease and at the last generation, a girl, child of one of the brothers affected, presented follicular atrophoderma and hypotrichosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

[Cutaneous carcinoma and papillomavirus 5. Discovered during prolonged UVA photochemotherapy].

INTRODUCTION: HPV 5 is an opportunistic oncogenic virus found mainly in patients with verruciform epidermodysplasia or in certain immunodepressed patients. CASE: We report the case of a 48-year-old patient who developed, over a 5 year period, 30 skin carcinomas after prolonged puvatreatment. HPV 5 was identified. The course has been stable under treatment with etretinate and alpha-interferon. COMMENTS: The role of ultraviolet irradiation as a carcinogenic agent is raised by this observation, both as a primary factor and as a local factor favouring immunosuppression and proliferation of opportunistic HVP.

Adult↗
PubMed1994

[Experimental evaluation of anti-seborrheic substances].

The effects of different drugs on the sebaceous gland is described by the use of an animal model. A castrated rat Wistar was made hyperseborrheic with implant of testosterone. On this model, graduated by a different anti androgen (acetate of cyproterone), anti-seborrheic effect is measured, bound or not the antiandrogenic effect of retinoids, cimetidine, spironolactone and, minoxidil. Retinoids reduced the surface of sebaceous glands but did not express an anti-androgenic effect. Cimetidine and spironolactone presented the same action. Minoxidil had no effect on this model.

Animals↗
PubMed1994

Photosensitive and phototriggered pityriasis rubra pilaris.

We report a 59-year-old man with photosensitive and photodistributed pityriasis rubra pilaris. The phototriggering of the lesions was obtained by the iterative polychromatic phototest (3 times the minimal erythema dose (MED) on 3 consecutive days) and was also observed on the supra MED sites, which began the 10th day and persisted several months. Our results are comparable to those already obtained in photosensitive psoriasis, which helped us to identify the clinical similarities of photosensitivity in these two instances of dermatosis.

Humans↗
PubMed1993

Cutaneous metastasis from follicular carcinoma of the thyroid gland.

A 65-year-old man with a history of malignant melanoma presented a reddish nodule on the left temporal region of the scalp. Histologic features and immunoperoxidase staining on the skin biopsy specimen confirmed the diagnosis of metastatic thyroid follicular carcinoma. The patient presented recurrences of metastatic skin nodules, lung and bone metastases, and died after a 3-year follow-up.

Adenocarcinoma, Follicular↗
PubMed1993

[Porphyria cutanea tarda and HIV-1 infection. 2 new cases].

We report two new cases of HIV-1 seropositive subjects who developed porphyria cutanea tarda (PCT). Laboratory examinations revealed a rise of uroporphyrin in the 24-hour urine and a fall of CD4 lymphocytes below 200/mm3 reflecting severe immuno-depression. Treatment with iterative phlebotomy resulted in regression of PCT. In both patients HIV seropositivity was discovered before PCT was diagnosed. Up to now, none of the hypotheses put forward concerning the exact role played by HIV in the genesis of PCT has been confirmed.

AIDS-Related Opportunistic Infections↗
PubMed1993

[Vulvar lichen sclerosus].

Lichen sclerosus's pathogeny, the most frequent vulvar dystrophy predominant at the start of menopause, is still enigmatic. Its repercussions on the functional level can be disabling. Its clinical sides include atrophy and sclerosis. The evolution of the past towards great atrophies (kraurosis vulvae) may today be prevented by early diagnosis and treatment (essentially dermocorticoïde). Even if the risk of degenerescence is low, it's not negligible and these patients must be put under steady surveillance.

Adrenal Cortex Hormones↗
PubMed1993

[Keratoderma palmoplantaris striata].

Keratoderma palmoplantaris striata is a rare disease, with a well-defined clinical presentation which differentiates it from other cutaneous keratoses. Although there is a familial incidence, sporadic cases have been reported. Extracutaneous lesions have also been observed. We report a new case which responded well to retinoid therapy.

Adult↗
PubMed1992

[Does bullous pemphigoid with negative direct immunofluorescence exist? Apropos of 3 cases].

Subepidermal autoimmune bullous dermatoses are defined by clinical, histological and immunological criteria, notably the presence of anti-basement membrane antibodies detectable in vivo by direct immunofluorescence. We report three cases where anti-basement membrane antibodies were not detectable in vivo by direct immunofluorescence but were detected in high titres by indirect immunofluorescence. This situation is extremely rare in the literature. The first case concerns a 69-year old woman seen for bullous and pruriginous lesions of the lower limbs of 2 months' duration. Histological examination found dermoepidermal bullae with, in their lumen, a serous fluid spotted with numerous polymorphonuclears. A search for anti-basement membrane antibodies was positive in significant titres (1,280; 320; 480) at indirect immunofluorescence on rabbit lip whereas five successive direct immunofluorescence test in perilesional skin and on the thigh medial surface remained negative. The second case is that of a 91-year old woman suffering from generalized pruritus associated with erythematous lesions predominant on the extension surfaces of the forearms and thighs, without any bullous lesion. Pathological examination only showed a superficial dermal lymphocytic infiltrate. Four direct immunofluorescence tests were negative, whereas a search for anti-basement membrane antibodies on rat oesophagus was positive at 1/1,280. The third case resembles the second one. It concerns a 72-year old woman who consulted for generalized pruritus of several months' duration which interfered with sleep and was incompletely relieved by emollients. There was no specific skin lesion. Pathological examination revealed nothing more than a discrete perivascular mononucleate infiltrate. Direct immunofluorescence tests performed on two occasions on the skin of the abdomen and of the medial thigh surface were negative.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗