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Biomedical subjects

L Bruckner-Tuderman

Publications and source records attributed to L Bruckner-Tuderman.

118 records · Page 7Linked to original sources
PubMed1988

Epidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen.

Epidermolysis bullosa acquisita (EBA) is a severe, chronic blistering disease of the skin. EBA patients have circulating and tissue-bound autoantibodies to a large (Mr = 290,000) macromolecule that is localized within the basement membrane zone between the epidermis and dermis of skin, the site of blister formation. The "EBA antigen" is known to be distinct from laminin, heparan sulfate proteoglycan, fibronectin, the bullous pemphigoid antigen, elastin, and collagen types I, II, III, IV, and V. Sera from patients with EBA, two monoclonal antibodies to the EBA antigen, and a monoclonal antibody to the carboxyl terminus of type VII procollagen identically label human amnion and skin by immunofluorescent and immunoelectron microscopy. Western immunoblots of the EBA antigen extracted from skin and of type VII procollagen labeled with the above sera and antibodies are identical. None of the sera or antibodies labels Western blots of pepsinized type VII collagen which is missing the globular amino and carboxyl terminal domains. These data show that the EBA antigen is the carboxyl terminus of type VII procollagen.

Amino Acid Sequence↗
PubMed1988

Acitretin in the symptomatic therapy for severe recessive x-linked ichthyosis.

Eight men with severe recessive x-linked ichthyosis were treated with acitretin, the main metabolite of etretinate, during four months. All of the patients showed marked clinical improvement of scaling during therapy. Hypervitaminosis A-type adverse reactions were observed in all patients. Although the overall tolerance was good, therapy was interrupted in one atopic patient because of pruritus. There were no undesirable laboratory changes in values. Thirty-five milligrams of acitretin daily provided the best efficacy, with minimal side effects. The beneficial effect of this retinoid lasted between four and six weeks after therapy was stopped. These results suggest that acitretin is a useful agent in the symptomatic therapy of severe recessive x-linked ichthyosis resistant to topical therapeutic modalities. Good results with this agent can be achieved with interval therapy adjusted to seasonal variations of the skin symptoms.

Acitretin↗
PubMed1988

[Andrologic studies in patients with X-chromosome recessive ichthyosis].

In the past there have been several reports of testicular abnormalities (hypogonadism, cryptorchidism and so-called atypical germ cells) in patients with recessive X-linked ichthyosis. Anamnestic interviews, clinical as well as chemical investigations in ten patients (aged 25-52 years) with recessive X-linked ichthyosis have been undertaken in order to examine their endocrine and exocrine testicular function. Normal serum levels of gonadotropins were observed in nine of ten patients, while four of ten men had decreased levels of dehydroepiandrosterone. In addition, in eight of these ten patients spermatological alterations were found: two patients suffered from oligozoospermia, while asthenozzospermia occurred in seven patients and teratozoospermia was registered in three men. Although andrological abnormalities were found in five out of ten patients (cryptorchidism, varicocele), the high incidence of spermatological alterations is remarkable. Further investigations will be necessary to clarify whether steroid sulphatase is responsible for some of these alterations, especially for the pronounced asthenozoospermia.

Adult↗
PubMed1987

Tissue form of type VII collagen from human skin and dermal fibroblasts in culture.

The triple-helical domain of type VII collagen was isolated from human placental membranes by mild digestion with pepsin, and polyclonal antibodies were raised in rabbits against this protein. After affinity purification the antibodies specifically recognized type VII collagen in both the triple-helical and the unfolded state. They also reacted with the fragments P1 and P2, derived from the triple-helical domain by further proteolysis with pepsin, but did not crossreact with other biochemical components of the dermal connective tissue. In skin the presence of a fragment of type VII collagen, similar to that isolated from placenta, was demonstrated by SDS-PAGE and immunoblotting. Type VII collagen represented less than 0.001% of the total collagen extracted by pepsin digestion from newborn or adult skin. The tissue form of type VII collagen was obtained from dermis after artificial epidermolysis with strongly denaturing buffers under conditions reducing disulfide bonds. The protein was identified by immunoblotting with the antibodies. The molecule was composed of three polypeptides with an apparent molecular mass of about 250 kDa, each. Similar large-molecular-mass chains could be identified by immunoblotting in extracts of human fibroblasts in culture.

Cells, Cultured↗
PubMed1987

Fibroblasts of an acrogeria patient produce normal amounts of type I and III collagen.

An 11-year-old patient with clinically typical acrogeria is presented. She had wrinkled 'old-appearing' acral skin, thin skin on the whole integument, grooved tip of nose, mottled hyperpigmentation of fold regions and thickened dystrophic toe nails. Histologically, the epidermis was flattened, dermis thinned and the connective tissue densely packed with very few cellular elements. Electron microscopic examination revealed partially abnormal collagen fibers with varying caliber, degenerated elastic fibers and dystrophic fibroblasts. The patient's fibroblasts produced normal amounts of apparently normal type I and III collagen as analyzed after radioactive labeling. Also, the levels of cytoplasmic mRNA for both of these collagens were similar in acrogeria and control cells.

Aging↗
PubMed1986

[Gluteal granuloma in an infant].

This report deals with a 5-month-old infant who at the age of 2 months was treated with topical fluorinated steroids for dermatitis in the diaper area. The treatment was continued for about 2 months. Some weeks later, multiple oval and round dark brown nodules developed in the inguinogenital areas. Histologic examination of these lesions revealed intense granulomatous infiltration in the upper dermis that consisted of lymphocytes, neutrophilic and eosinophilic granulocytes, and histiocytes. After steroid therapy was discontinued, the nodules slowly disappeared.

Buttocks↗
PubMed1985

[Collagen in current connective tissue research. Application of molecular biological methods to medical problems].

Collagen plays an important role in connective tissue diseases. It is the most abundant protein of the human body and serves as a model protein in the study of a variety of diseases. At present, at least ten different genetically distinct collagen types are known and characterized to at least some extent. In patients with Ehlers-Danlos syndrome, Marfan syndrome and osteogenesis imperfecta, molecular defects in the type I collagen genes are already known. It is very likely that in the near future vast amounts of data about the collagens, collagen defects and their involvement in human pathophysiology will become available.

Chemical Phenomena↗
PubMed1985

Unusual cutaneous dissemination of a tropical fish tank granuloma.

A patient with an unusual disseminated tropical fish tank granuloma is presented. The infection manifested first as red, subcutaneous nodules of the right hand and forearm. During subsequent days and weeks, nodules appeared on the left arm, on the ventral and dorsal thorax, on one leg, and the face. The diagnosis was based on, in addition to the history and the clinical aspects, a positive reaction to intracutaneous testing with a specific Mycobacterium marinum antigen and on the histologic examination of biopsy specimens. The larger nodules were treated with excision. Smaller nodules disappeared spontaneously after the larger ones had been surgically removed.

Aged↗