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Biomedical subjects

M Fartasch

Publications and source records attributed to M Fartasch.

At least 73 records · Page 4Linked to original sources

Recent epidemiological and genetic studies in atopic dermatitis.

In a prospective computerized study, basic and minor features of atopic dermatitis were studied systematically in established cases of atopic dermatitis (AD; n = 428) and compared with subjects randomly collected from the caucasian normal population of young adults (NP; n = 659). Complete genetic data (history of AD, allergic rhinitis, allergic asthma) were obtained from the first-degree relatives of all subjects (about 9,000 family members). In young adults, atopy was found in 22.5% (AD 4.7%, allergic rhinitis 17.9%, allergic asthma 4.8%). Of 428 AD patients, 54% had 'pure' AD and 46% suffered from a 'mixed' type with concomitant respiratory allergies (RA). The general risk of developing AD and atopy increases with each first-degree family member already suffering from atopy. Our study further supports the evidence of a genetic influence on symptom specificity. Risk figures for genetic counselling are given. The complex interplay of atopic symptoms and signs in the diagnosis of AD has been analysed by a CART analysis. Compared with non-eczematous controls, the odds ratios (OR) of frequent features in AD are as follows: xerosis (OR 27.9, 95%-CI 23.2-33.8), itch when sweating (OR 25.4, 95%-CI 21. 1-30.1), white dermographism (OR 19.3, 95%-CI 16.2-23.2), wool intolerance (OR 15.8, 95%-CI 13.40-18.5), whereas the OR of elevated IgE (> 150 U/ml) was only 5.0 (95%-CI 4.3-5.8). But when comparing the AD patients with concomitant RA separately, the odds ratio is increased to 16.2.

Adult↗

The barrier function in atopic dry skin. Disturbance of membrane-coating granule exocytosis and formation of epidermal lipids?

Non-eczematous atopic dry skin (DS) shows an enhanced transepidermal water loss denoting an impaired water permeability barrier (WPB) function. The WPB is formed by intercellular lipid lamellae located between the horny cells of stratum corneum (SC). The lipids are provided via the exocytosis of membrane-coating granules (MCG). By differentiating two dynamic states of MCG, the ultrastructural morphometric comparison of atopic DS and healthy skin of controls revealed a retarded and incomplete extruding mechanism of these organelles. Additionally the structure and spacial organization of the epidermal lipids in DS and healthy skin were visualized and analysed by applying a special primary fixation (acrolein vapour) and post-fixation with ruthenium tetroxide. The present findings suggest that some pathologic extruding mechanism of MCG in DS may be responsible, at least partly, for the recently detected biochemical alterations of epidermal lipids and for the deficient WPB.

Adult↗

Immunohistochemical and ultrastructural study of histiocytosis X and non-X histiocytoses.

The diagnostic reliability of ultrastructural and immunohistochemical examinations on routinely processed biopsy specimens of cutaneous histiocytic proliferations (histiocytosis X, n = 7; juvenile xanthogranuloma, n = 4; necrobiotic xanthogranuloma, n = 2; traumatic granuloma of the tongue, n = 1) was evaluated. S-100 protein, peanut agglutinin, and the antibody Mac-387 were used as markers for histiocytes. The frequency of Birbeck granule-containing cells in seven histiocytosis X lesions did not correspond with the number of S-100+ or peanut agglutinin+ cells. All neoplastic histiocytosis X cells were positive for S-100 protein and peanut agglutinin but were negative for Mac-387. Histiocytes of juvenile xanthogranuloma, necrobiotic xanthogranuloma, and traumatic granuloma were strongly positive for Mac-387 but were negative for S-100 protein and peanut agglutinin, except for the peanut agglutinin-reactive Touton giant cells. Mac-387 reliably differentiates histiocytic proliferations of the monocyte/macrophage system from those of the dendritic cell system. For the diagnosis of histiocytosis X, both S-100 protein and peanut agglutinin positivity in histiocytes is as reliable as ultrastructural demonstration of Birbeck granules.

Adult↗

Abnormalities of keratinocyte maturation and differentiation in keratosis palmoplantaris striata. Immunohistochemical and ultrastructural study before and during etretinate therapy.

Keratoderma striatum (Brünauer-Fuhs type) with linear keratotic elevations on the palms and small islets (areata form) on the soles is a rare form of palmoplantar keratoderma (PPK). An immunohistochemical and ultrastructural study has been performed to characterize the altered keratinization and maturation patterns in this disease before and during complete clinical remission on therapy with etretinate. Anticytokeratin antibody KL1 showed no significant difference in reaction pattern either between healthy controls and PPK or following therapy. Earlier expression of both filaggrin and involucrin was found in PPK in comparison with the controls. During etretinate therapy the filaggrin pattern returned to normal, whereas the altered involucrin pattern was not influenced. Ultrastructural investigations before treatment revealed tightly packed tonofibrils (TF) and large masses of keratohyalin (KH) granules with abnormal configuration. During therapy the TF and KH granules were reduced in number and size. KH granules now showed frayed borders. Moreover, a transitional cell zone, focal parakeratosis with lipid droplets, and dyskeratotic cells became apparent. The normalization of filaggrin pattern accompanying the clinical remission of these lesions implies a role of this keratinocyte differentiation protein in the pathogenesis of these lesions. Since etretinate is assumed to act at a very late stage of epidermal differentiation, there was no influence on the altered expression of involucrin during etretinate therapy. Despite the clinical remission, fine structural abnormalities persisted, indicating that the deviations from the normal keratinocyte differentiation program in PKK occur very early.

Adult↗

Mineralization of collagen and elastic fibers in superficial dystrophic cutaneous calcification: an ultrastructural study.

The ultrastructural morphology of localized skin calcifications without associated diseases and with normal serum calcium and phosphate ion values is still unknown. In a case of superficial dystrophic calcinosis cutis (DCC), the role of collagen, elastin and ground substance in the process of calcification and the organization of the apatite crystals could be studied by light and electron microscopy despite technical difficulties in sectioning the hard tissue. Ultrastructural investigation revealed the nucleation of calcification being related to collagen and elastic fibers. No intracellular calcification was found. A flower-like arrangement of pleomorphic crystals was found around single collagen fibrils resembling the calcification of collagen seen in bone tissue. The elastic fibers showed a different pattern of calcification compared with other diseases (e.g. pseudoxanthoma elasticum) with known calcification of the elastic fibers. The process of mineralization was initially linked to the microfibrils of the elastic fiber.

Adult↗

[Hyalinosis cutis et mucosae (Urbach-Wiethe) in 2 sisters].

The authors report on 2 sisters who had suffered from both atopic eczema and hoarseness since their birth. Rough, yellowish-white papular deposits in the skin and the oral mucosa had developed during their childhood. The clinical diagnosis of hyalinosis cutis et mucosae was confirmed in both cases by means of histology and electron microscopy.

Adult↗

[Characteristics of polymorphous light dermatosis--results of a prospective survey and study of 302 affected patients].

In a prospective study on 302 patients (females = 87%), we registered the historical and clinical data of polymorphous light eruption (PLE) and compared our results to similar studies from Finland, Sweden, and the USA. The mean age of onset of the disease was 24.0 years; the average duration time was 10.1 years. We found the following clinical and historical characteristics of PLE: skin types I and II - 49%; positive family history - 29%; latent period between sun exposure and the first skin eruptions - in 39% less than 2 hours, in 5% 3 days and more. Mostly we observed paular eruptions and strong pruritus, and usually the typical body areas were involved (i.e. décolleté, upper and lower arm, thigh, back of the hand, and face). In 64% of the patients who avoided exposure to the sun, the PLE did not continue but less than a week. Our findings may supply a useful tool regarding the diagnosis of ambiguous photodermatoses.

Adolescent↗

Atopic dermatitis--ichthyosis vulgaris--hyperlinear palms--an ultrastructural study.

Some 30-50% of cases of atopic dermatitis (AD) are believed to be associated with autosomal dominant ichthyosis vulgaris (ADI). The diagnosis of ADI can be proved by the ultrastructural demonstration of fewer and abnormal keratohyalin (KH) granules in all ADI patients, even in clinically unaffected skin. To prove the suggested frequent association of ADI with AD, an ultrastructural investigation of dry skin of 49 AD patients was performed. Only in 2 (4%) patients ADI could be confirmed by electron microscopy. In 17 patients, including the 2 patients with abnormal KH, hyperlinear palms were clinically seen. The present study yields evidence that hyperlinear palms, if present, and dry skin are in most cases a phenotypic marker of AD and not a sign of concomitant ADI. A histologically absent stratum granulosum in AD does not signify by itself a manifestation of concomitant ADI.

Adolescent↗

Are hyperlinear palms and dry skin signs of a concomitant autosomal ichthyosis vulgaris in atopic dermatitis?

In 30% to 40% of cases atopic dermatitis (AD) is believed to be associated with autosomal dominant ichthyosis vulgaris (ADI). The diagnosis of ADI can be proved by the ultrastructural demonstration of a defective keratohyalin (KH) synthesis, resulting in minute granules of crumbly appearance in only one layer of granular cells. To investigate the suggested frequent association of ADI with AD, ultrastructural examination of dry skin of 49 AD patients was performed. Only in 2 patients abnormal KH was demonstrated by electron microscopy. 17 patients, including the 2 patients with abnormal KH, showed hyperlinear palms. The present study shows that hyperlinear palms and dry skin are in most cases a phenotypic marker of AD alone and not a sign of concomitant ADI. A histologically one-layered or absent stratum granulosum may occur in the dry skin of patients with only AD and does not indicate a manifestation of concomitant ADI in all cases.

Adolescent↗

Evaluation and relevance of atopic basic and minor features in patients with atopic dermatitis and in the general population.

In a prospective computerized study on atopic dermatitis (AD) several basic and minor clinical features in patients with AD (n = 110) and a sample of the normal population (n = 527) was studied systematically and analysed statistically with regard to their diagnostic importance. On basis of chi-square values a diagnostic score system was constructed which might help to establish a firm diagnosis of AD in patients with ambiguous cutaneous inflammatory disease. Based on this score system patients with more than 10 points should be considered atopic, patients with 6 to 10 points are suspected to be atopics. An association between serum IgE and the amount of atopic points was found. Seven percent of the normal population sample proved to be obviously atopic, another 19% were suspected to be atopics.

Adult↗

Cutaneous lymphoepithelioid lymphoma (Lennert's lymphoma). Combined immunohistological, ultrastructural, and DNA-flow-cytometric analysis.

Lymphoepithelioid lymphoma (LEL) is a peculiar type of low grade malignant T cell lymphoma usually arising in lymph nodes, characterized immunohistologically by predominant T-helper/inducer lymphocytes intermingled with clusters of epithelioid cells. An uncommon case of LEL with cutaneous involvement is reported with additional reference to ultrastructure and DNA-flow-cytometric analysis (DNA-FCM) of the lymphoma. Light microscopy showed subepidermal bandlike infiltrates of lymphocytes and clusters of epithelioid cells extending into the subcutaneous tissue. By immunohistochemistry the presence of a high percentage of T-helper/inducer lymphocytes was confirmed. DNA-FCM demonstrated an aneuploid cell population indicating malignant cells. Our results are in accordance with earlier ones established in lymph nodes.

Aged↗

[Polyvinylpyrrolidone dermatoses. Clinical aspects and ultrastructural morphology].

High-molecular polyvinylpyrrolidone (PVP) is used in order to induce a depot of subcutaneously or intramuscularly applied drugs. Deposits of PVP in skin plaques persisting for years and distant from the injection site have been demonstrated by the use of a modified fixation and embedding method for electron microscopy. The storage of PVP did not only occur in macrophages but also in other dermal cells whose pinocytotic activity seems to have been underestimated so far. PVP was found in mucinous and serous cells of sweat glands, in myoepithelial cells, endothelial cells, mast cells, and in the perineurium as well as in Schwann cells.

Delayed-Action Preparations↗

Disseminated cicatricial pemphigoid in a child and in an adult. Ultrastructural diagnostic criteria and differential diagnosis with special reference to acquired epidermolysis bullosa.

The first case of an infant affected with a rare, disseminated variant of benign cicatricial pemphigoid is described, showing the same ultrastructural features of initial blister formation as an adult patient. These consist in edematous changes within the superficial dermis caused by vesiculation or dissolution of cellular and noncellular connective tissue elements, coalescing into subepidermal blisters. Differential diagnosis excludes other nonhereditary bullous disorders because of the ultrastructure of the dermo-epidermal junction in nascent blisters and in perilesional skin. In spite of evident clinical, histological, and immunohistological similarities as well as controversial and confusing immunological studies, acquired epidermolysis bullosa can be clearly separated from our case by a diagnostic hallmark on the electron-microscopical level, i.e., band-like IgG depositions beneath the basal lamina. This is demonstrated in comparing the two cases of disseminated cicatricial pemphigoid with three patients suffering from acquired epidermolysis bullosa, thus providing evidence that disseminated cicatricial pemphigoid and acquired epidermolysis bullosa are two distinct nosologic entities.

Adult↗

Disseminated cicatricial pemphigoid.

A patient is presented with generalized blistering and scarring eruptions, showing immunopathological, histological and electron microscopical characteristics of disseminated cicatricial pemphigoid, successfully treated by combined therapy of systemic steroids and azathioprine.

Azathioprine↗

[Coincidence of light urticaria and lichen ruber planus].

A 49-year-old patient with concomitant solar urticaria (SU) and lichen planus (LP) is presented. The sunlight- and UV-A-provoked wheal and flare SU reactions were interpreted as an antigen-antibody reaction, probably involving a reaginic antibody, because SU was passively transferred by a serum factor utilizing the Prausnitz-Küstner reaction. The reduced percentage of suppressor cytotoxic T-cell subset in peripheral blood typical of active LP may facilitate the increased production of reaginic antibodies after presentation of antigen by sunlight. Not only was the usual immunological reaction of LP (similar to graft-versus-host reaction) suppressed by PUVA treatment, but increased tolerance to sunlight was also achieved.

Biopsy↗

[Congenital Langerhans-cell histiocytosis presenting as a varicella infection].

UNLABELLED: We report on a preterm infant (33rd gestational week) with a varicella-like congenital rash, which initially appeared to respond to therapy with acyclovir. At the age of 3 weeks, lesions were in different stages of evolution and still resembled a varicella zoster virus (VZV) infection. However, since proof of VZV infection was lacking and new lesions erupted at the age of 4 weeks, a skin biopsy was performed which revealed a diagnosis of Langerhans cells histiocytosis. Therapy with prednisone resulted in prompt healing of the lesions. DISCUSSION: Congenital Langerhans cell histiocytosis is rare and symptoms may vary substantially from case to case. Like in our observation it may be confused with congenital varicella. In case of congenital skin lesions of uncertain etiology a skin biopsy should be performed.

Anti-Inflammatory Agents↗