[Dosage and modulation of cutaneous steroid receptors during antihormonal therapy in a case of Stewart-Treves syndrome]. / Dosage et modulation des récepteurs stéroïdiens cutanés sous thérapeutique anti-hormonale dans le cas d'un syndrome de Stewart-Trèves.

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)

Collagen biosynthesis and isomorphism in a case of Ehlers-Danlos syndrome type VI.

Collagen metabolism was studied in fibroblasts grown from a skin biopsy specimen of a patient who presented the striking clinical features of Ehlers-Danlos syndrome and, in particular, hyperextensibility of the skin, hypermobility of the joints, and kyphoscoliosis. A reduction in lysine hydroxylation, characteristic of Ehlers-Danlos Type VI, was observed after labelling of the collagen with 14C-proline and 3H-lysine. Other modifications in the collagen metabolism of fibroblast cultures were noted, including an increase in collagen and total protein synthesis, and an increase in both the Type I and Type III collagen. The percentage of Type III collagen was, however, lower than in the control fibroblasts. The results point out the complexity of collagen disturbances in Ehlers-Danlos Type VI.