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Biomedical subjects

O Braun-Falco

Publications and source records attributed to O Braun-Falco.

At least 19 recordsLinked to original sources

In situ localization of IgG in epidermolysis bullosa acquisita by immunogold technique.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is an immunologically mediated mechanobullous dermatosis. Recently, serum antibodies in patients with EBA have been reported to bind to the carboxyl terminus of type VII collagen, the disease antigen. However, the precise localization of immunoglobulins in diseased skin has not been demonstrated. OBJECTIVE: In the present study skin samples taken from a 59-year-old man with EBA were processed for immunoelectron microscopy. METHODS: A preembedding immunogold ultrastructural technique was applied. RESULTS: Gold particles, indicative of IgG binding, were deposited within the anchoring plaques and within the lamina densa in some perilesional skin sections. In a similar distribution, IgG-containing amorphous aggregates were detected within the basement membrane zone of perilesional and lesional skin. In some places these immunoglobulin deposits covered the whole region of anchoring fibrils. CONCLUSION: These results demonstrate the precise localization of IgG within the basement membrane zone of EBA skin that corresponds to the carboxyl terminus of collagen VII in normal skin.

Diagnosis, Differential

Ultrastructural immunogold studies in two cases of linear IgA dermatosis. Are there two distinct types of this disease?

It has been suggested that patients with homogeneous linear IgA deposits at the basement membrane zone constitute a distinct bullous disorder called linear IgA dermatosis (LAD) of adults or children. The results of the present ultrastructural immunogold study in two patients with LAD suggest that LAD is not a single disease entity. LAD in a 10-year-old girl was found to be ultrastructurally similar to an IgA-type pemphigoid. IgA was detected in the uppermost lamina lucida underlying the basal cell plasma membrane. In a second patient, an 86-year-old man, IgA deposits were present within the lamina densa and the anchoring plaques. The distribution of IgA in this patient was ultrastructurally identical with that of IgG in epidermolysis bullosa acquisita skin and with that of the non-collagenous globular terminus of collagen VII within the basement membrane zone of normal skin. By using the immunogold technique, we could distinguish two distinct types of LAD according to the IgA binding sites in the diseased skin. We suggest that different labelling patterns may correspond to different clinical pictures.

Aged

Treatment of systemic sclerosis with gamma-interferon.

Numerous drugs have been recommended for the treatment of systemic sclerosis, but without any significant effect on the fibrotic stage of this disorder. Because recombinant gamma-interferon (gamma-IFN) is a potent and selective inhibitor of fibroblast proliferation and collagen production by human dermal fibroblasts in vitro, we assessed the effects of gamma-IFN treatment on the skin and on pulmonary function in patients with systemic sclerosis. Fourteen patients entered the study, and nine completed the 12-month trial. Fifty micrograms/day of gamma-IFN was administered subcutaneously 3 days per week. At the end of the 12-month treatment period a significant improvement was observed in total skin score, and blood gas analysis showed a significant increase in Pa O2 during therapy with gamma-interferon. Other clinical parameters (dysphagia, Raynaud's phenomenon, cardiac involvement) were not altered significantly. No serious adverse effects were noted. These results suggest a beneficial effect of gamma-IFN on the cutaneous fibrotic abnormalities and on lung fibrosis in systemic sclerosis.

Adult

Ultrastructural binding sites of endomysium antibodies from sera of patients with dermatitis herpetiformis and coeliac disease.

The ultrastructural binding sites of endomysium antibodies, specific serological markers of gluten sensitive enteropathy, were investigated in the rabbit oesophagus using the immunogold technique. Endomysium antibodies from sera of patients with dermatitis herpetiformis and with coeliac disease bound in an identical manner in a non-fibrillar material closely associated with fine collagenous-reticulin fibrils and also with similar fibrils connecting smooth muscle cells and elastic tissue in the endomysial connective tissue. These observations suggest that IgA antibodies in sera from patients with dermatitis herpetiformis and coeliac disease recognise a common antigen in an amorphous component associated with the reticular connective tissue of oesophageal lamina muscularis mucosae and thus confirm the probable identity of IgA class endomysium and jejunal antibodies.

Animals

[Extracutaneous malignant melanomas: clinical aspects and biology].

Approximately 5% of all malignant melanomas originate from primarily non-cutaneous sites. They are preferentially distributed along the choroid, the meninges, the subserous space of the oesophagus and the intestinal tract, at transitional areas of mucous membranes, and along organ capsules, the periadventitial tissues of major blood vessels, and, occasionally, muscle fasciae. Little is known about their biological behaviour; the prognosis, however, generally has to be considered less favourable than that of primary cutaneous malignant melanomas, probably at least in part because they are often not discovered until they are in advanced stages. There are no specific clinical signs suggestive for non-cutaneous malignant melanomas; symptoms vary with the site of manifestation of the tumours. Interpretation of these clinical data based on results from comparative anatomical studies leads to the conclusion that extracutaneous malignant melanomas in humans are residuals of phylogenetically old, extensive and well-developed non-cutaneous pigment cell patterns demonstrable in a variety of lower vertebrates, which result from interactions between organogenesis and pigment cell development during ontogeny. This demonstrates that consideration of fundamental principles of developmental biology can give diagnostic clues to the localizations at which primary non-cutaneous malignant melanomas may be suspected and can thus facilitate decisions about what diagnostic procedures are necessary.

Animals

[Erosive pustular dermatosis of the scalp after zoster ophthalmicus and trauma].

Erosive pustular dermatosis of the scalp (EPDS), first described in 1979, is a rare, chronic, pustular condition with scarring alopecia, and nonspecific histological findings. While the initial responded to steroids is good, it can be treated successfully by oral administration of zinc sulphate. Local trauma has recently been suggested to play a role in the pathogenesis of EPDS. The differential diagnosis of EPDS includes folliculitis decalvans, sterile eosinophilic pustulosis Ofuji, pustular psoriasis vulgaris, trichophytosis, Perifolliculitis capitis abscedens et suffodiens, pemphigus vulgaris and cicatricial pemphigoid. We present the cases of a 74-year-old woman suffering from EPDS following herpes zoster ophthalmicus and of a 54-year-old man in whom EPDS followed a head injury.

Aged

[Erythema nodosum and non-Hodgkin lymphoma].

A 36-year-old woman presenting with erythematous painful tender inflammatory nodular lesions on both knees, shins and ankles is reported. Erythema nodosum (EN) was diagnosed. Further clinical examination revealed hilar lymphadenopathy. A lymph node was removed for histological examination, and a centroblastic non-Hodgkin lymphoma was diagnosed. Remarkably, in this patient EN occurred before the haematological malignancy was detected.

Adult

[Multiple primary malignant tumors in patients with malignant melanoma of the skin].

The incidence of further malignant tumors was analyzed by means of a retrospective study in 2335 melanoma patients. During the follow up 85 patients (3.64%) developed a second malignant tumor: 29 patients multiple primary malignant melanomas (mm), 49 a mm and a further nonmelanocytic malignant tumor, and 7 multiple primary mm and an additional non-melanocytic malignant tumor. Statistical calculations revealed that female melanoma patients have a 20 fold, and male melanoma patients a 30 fold increased risk to develop further malignant melanomas. In contrast, there was no increased incidence of non-melanocytic malignant tumors, breast carcinoma included. A total skin examination has therefore to be performed during follow up investigations of melanoma patients for early detection of further malignant melanomas.

Adult

Prognostic significance of DNA cytometry in cutaneous malignant lymphomas.

The current classification of cutaneous malignant lymphomas (ML) into low-grade and high-grade lymphomas was found to be of limited reproducibility and permitted only a rough prediction about outcome. With this in mind, the relationship between nuclear DNA content and both prognosis and histologic grading according to the Kiel classification was evaluated on Feulgen-stained imprint specimens. In all, 49 cases of malignant non-Hodgkin's lymphoma, primary of the skin or with an involvement of the skin as one of the first symptoms, were studied using a computerized high-resolution image analysis system. The 2c deviation index (2cDI), which reflects the variation of the nuclear DNA values around the normal diploid peak, was found to be the best prognostically relevant criterion. Using the 2cDI, a significant discrimination (P less than 0.001 in the U test) between low-grade and high-grade ML was achieved. The prognostic benefit of the 2cDI was well documented by a significant inverse correlation between the 2cDI and the period of time until the patients progressed at least into one higher stage or died of lymphoma (r equals -0.63, P less than 0.05). In addition, the 2cDI enabled prognosis of the course of disease. In the group with low 2cDI values (2cDI, less than 0.5), no progression of the disease was observed after 1 year. In the groups presenting with a 2cDI between 0.5 and 1.0 and higher than 1.0, a progression was found in 57% and 64% of the cases studied, respectively. In conclusion, these measurements indicate that the determination of DNA distribution patterns in imprint specimens allows a precise and objective prognostic evaluation of cutaneous ML.

Adult

[Are allergies really increasing?].

A presumed rise in the prevalence of atopic diseases in industrialized countries is currently subject to extensive discussion. Due to a lack of epidemiological longitudinal studies, the controversy among the experts was based mainly on speculation. Only recently have data from various European countries confirmed an increase in atopic diseases. Studies from Sweden, Switzerland and Denmark showed a definite increase in atopic diseases. Studies from Sweden, Switzerland and Denmark showed a definite increase in the prevalence of hay fever, bronchial asthma and atopic eczema, respectively. Japanese investigators have concerned themselves with the putative role of air pollutants in this development. They were able to show a relationship between exposure to particulate air pollutants (diesel exhaust) and the prevalence of mountain cedar pollinosis. To date a lack of baseline data has hampered the evaluation of epidemiologic trends in Germany. We performed a pilot survey study involving more than 1000 pre-school children that yielded initial evidence of an unexpectedly high prevalence of allergic conditions in Bavaria. A comparison with current epidemiologic data from other European countries suggests an increase in allergies in Germany similar to that observed elsewhere.

Cross-Sectional Studies

Ultrastructural basis for antigen mapping using sodium chloride-separated skin.

In many cases of autoimmune blistering skin diseases indirect immunofluorescence with serum of patients on 1 M NaCl-separated skin represents a rapid diagnostic tool before the use of more complicated immunoelectron microscopy. The present study demonstrates that in skin samples from five adults, separated using 1 M NaCl, 0.15 M NaCl and 0.01 M phosphate buffered saline (PBS), the split formed within the lamina lucida at an identical ultrastructural level. The sub-basal dense plate (SDP) with a wreath of anchoring filaments remained on the epidermal side of the split adjacent to the hemidesmosomal part of the plasma membrane of basal keratinocytes. The base of the split blister was constituted from the lamina densa, with a remote possibility of some anchoring filaments attached. We demonstrate that antigens on the roof of the NaCl- or PBS-split blister may be associated, beside intracellular hemidesmosomal structures, with the SDP and basement membrane components between the SDP and the basal keratinocytes as well as with anchoring filaments attached to the SDP. The observations reported here allow a more precise mapping of antigen determinants in blistering skin diseases.

Animals

Annular erythema associated with Sjögren's syndrome: a variant of systemic lupus erythematosus.

We present a Burmese patient with widespread annular erythema associated with Sjögren's syndrome. Unlike previously described cases, the disease occurred in the setting of systemic lupus erythematosus. Photoprovocation testing revealed light sensitivity in the UVA range with elicitation of subacute cutaneous lupus erythematosus-like lesions. The presence of an erythema annulare centrifugum-like eruption should initiate the search for Sjögren's syndrome and systemic lupus erythematosus.

Adult

Absence of extracellular enzyme activity and cytotoxicity in cell-free culture filtrates of Haemophilus ducreyi.

Ten isolates of Haemophilus ducreyi, including seven clinical isolates and three laboratory reference strains, were assessed for their ability to produce extracellular enzymes which might contribute to their pathogenicity. Protease, elastase, lecithinase, lipase or collagenase enzyme activity were not detected in culture filtrates from any of the isolates tested using either plate or spectrophotometrical assays. Furthermore, cell-free culture filtrates did not exhibit cytotoxic activity in vitro when tested using either an established tissue culture cell line (Vero) or a primary cell culture of human keratinocytes. These results suggest that extracellular products do not play a role in host invasion or production of tissue damage by H. ducreyi.

Cell-Free System