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Biomedical subjects

A Puissant

Publications and source records attributed to A Puissant.

At least 37 records · Page 2Linked to original sources
PubMed1988

[Dosage and modulation of cutaneous steroid receptors during antihormonal therapy in a case of Stewart-Treves syndrome].

We report a case with clinical symptoms of a Stewart-Treves syndrome 2 years after homolateral mastectomy for carcinoma. The mammary origin of the syndrome was found at histology and confirmed by assays of hormone receptors in skin biopsies before and during anti-hormone therapy. This treatment rapidly attenuated the clinical signs of both the lymphedema and the pseudo-Kaposi cutaneous lesions. Hormone receptor determinations are suggested as useful tools for the diagnosis and for surveillance of therapeutic efficacy. CASE HISTORY. This 68-year old patient whose right breast was removed in April, 1985 for scirrhous carcinoma was hospitalized in February, 1987 for lymphedema, pain and functional impotence of the right arm accompanied by Kaposi-like skin lesions in the homolateral mammary region, the right shoulder and the upper third of the right arm. Tamoxifen administration in doses of 30 mg/day was started on the day of the first biopsies at sites of the cutaneous lesions and continued for 60 days. Additional biopsies were taken on days 30 and 60 of therapy. Under therapy clinical improvement was noted with decrease in the lymphadenopathy and in the size of the nodular skin lesions. PATHOLOGY AND STEROID HORMONE ASSAYS. Standard histological studies were performed on one of the biopsies. The remaining tissues were flash frozen and assayed for estrogen and progesterone receptors. ER was determined by radio-ligand assay (RLA) to measure the free binding sites of the hormone or anti-hormone and by enzyme immunoassay (EIA) to detect both free and occupied antigenic sites.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenocarcinoma↗
PubMed1988

Papillon-Lefèvre syndrome. Ultrastructural study and successful treatment with acitretin.

Four siblings affected by Papillon-Lefèvre syndrome (PLS) ranged in age from 2 to 11 years. The parents were unaffected and parental consanguinity was present. The 2-year-old girl showed the early manifestations of PLS; that is, slight gingival swelling and erythema occurring simultaneously with minimal scaling of palms and soles. The other siblings, aged 5, 8, and 11 years, showed severe periodontopathy with tooth loss and marked palmoplantar keratoderma with a centripetal extension of the keratoses to the limbs and trunk. These three older siblings were treated with acitretin (Ro 10-1670), the free acid of etretinate, with complete clearing of the skin and healing of gingival pockets. Treatment was given for 16 months; teeth that erupted during therapy were free of periodontopathy and remained firmly anchored to the alveolar bone. In two of the children ultrastructural examination of involved skin was performed before and during acitretin treatment. Before treatment a large number of lipidlike vacuoles were found in corneocytes and in granulocytes; tonofilaments were reduced in number, and keratohyaline granules frequently showed a rectangular or globular shape. During treatment with acitretin these abnormalities diminished markedly. Thus, etretin is effective in treating PLS and, if treatment is started at an early age, should allow patients with PLS to have normal adult dentition.

Acitretin↗
PubMed1988

Pachydermoperiostosis with gastric hypertrophy, anemia, and increased serum bone Gla-protein levels.

We evaluated a patient in whom pachydermoperiostosis occurred in conjunction with anemia and gastric hypertrophy. The mechanism of the anemia appears multifactorial because, besides a myelofibrosis, a serum inhibitor of the late stage of erythropoiesis was detected. The elevated serum bone Gla-protein (osteocalcin) favors the hypothesis that primary hypertrophic osteoarthropathy represents an imbalance between increased osteoblastic bone formation and normal bone resorption.

Adult↗
PubMed1988

[Cutaneous localizations of disseminated Histoplasma capsulatum histoplasmosis in a case of acquired immunodeficiency].

A widespread maculo-papular cutaneous rash appeared on a HIV-positive young bisexual Cambodian man. He was treated for Mycobacterium tuberculosis and Pneumocystis carinii infections. He had been residing in France for seven years. Histology showed, within the dermis, abundant extracellular and intramacrophagic yeast-like organisms suggestive of histoplasmosis. Cultured specimens produced a growth of colonies after three weeks on Sabouraud 4 p. 100 dextrose agar at 25 degrees C. Numerous macroconidia were found which made the species diagnosis of Histoplasma capsulatum possible. Despite initiation of therapy with amphotericin B the patient died. Cutaneous involvement with or without specific features is uncommon in disseminated histoplasmosis. The specific cutaneous lesions are protean. They rarely are the presenting sign of initial infection. Disseminated histoplasmosis has a poor prognosis in acquired immunodeficiency syndrome: amphotericin B is not curative. Maintenance suppressive therapy with ketoconazole has been recommended following amphotericin B completion, although break-through has been reported.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Necrobiotic xanthogranuloma with myeloma. A case report.

Necrobiotic xanthogranuloma is a newly recognized cutaneous manifestation associated with paraproteinemia. Necrobiotic xanthogranuloma is distinctive clinically by yellowish-red nodular lesions, often ulcerated, with predilection for the periorbital area and ocular involvement. Histologically, it is characterized by the combination of inflammatory xanthogranulomas together with areas of necrobiosis. These skin lesions are associated with a dysglobulinemia and preceded the development of an IgG lambda myeloma in a previous patient. Hyperlipidemia may be found in some patients. A case of necrobiotic xanthogranuloma is reported, occurring in a 46-year-old man and associated with an IgG kappa myeloma.

Granuloma↗
PubMed1987

Clinically specific type of focal palmoplantar keratoderma with sensorineural deafness: an entity.

A case of focal palmoplantar keratoderma with associated sensorineural deafness is reported. The palmoplantar keratoderma was sharply marginated by geometric borders, spared the plantar arch and was accompanied by marked hyperkeratosis of palmar flexural creases. This syndrome, consisting of a very particular type of palmoplantar keratoderma associated with sensorineural deafness, appears to be distinctive enough to be considered as a clinical entity.

Adult↗
PubMed1987

[Antenatal diagnosis of hereditary epidermolysis bullosa].

Among the genetic disorders of the skin, the heterogeneous group of epidermolysis bullosa includes some of the most severe. Prenatal diagnosis is of considerable importance to the families who have had an affected child, or in which one of the parents is affected. The prenatal diagnosis is performed using fetoscopy and fetal skin biopsy. Fetal skin samples are taken at 20 weeks of gestation and are examined by light and electron microscopy to determine whether the fetus is affected. We report here the French experience on prenatal diagnosis of the severe inherited epidermolysis bullosae. Given the severity and frequency of Herlitz syndrome, it is not surprising that this is the most frequently encountered disease in our series of prenatal diagnosis (14 of 21 epidermolysis bullosae), followed by Hallopeau-Siemens (6 cases), and Pasini type (1 case). Our exclusion diagnosis of a Pasini fetus was the first prenatal diagnosis of this type of epidermolysis bullosa performed and reported in the literature. We stress here in this paper that observing the site of separation in the epidermal dermal junction is not sufficient to make a positive prenatal diagnosis. Prenatal diagnosis depends on the observation of the specific ultrastructural marker of the disease such as: hypoplasia and absence of hemidesmosomes and sub-basal dense plate in junctional epidermolysis bullosa-Herlitz, collagenolysis in recessive dystrophic epidermolysis bullosa-Hallopeau-Siemens, and absence and hypoplasia of anchoring fibrils in dominant dystrophic epidermolysis bullosa-Pasini. Until biochemical defects are clarified and suitable tests become available, electron microscopy remains the only current means for reliable, genetically useful, diagnosis of epidermolysis bullosa. In 62 per cent of cases of our series a prenatal diagnosis of exclusion of disease was made and we would stress that in high risk families repeated fetoscopies for prenatal diagnosis are possible in consecutive pregnancies thus allowing the family to have only normal children.

Epidermolysis Bullosa↗