[Chronic bullous disease of childhood. Long-term therapy over 8 years with 4,4'-diaminodiphenylsulfone]. / Chronisch bullöse Dermatose des Kindesalters. Langzeittherapie über 8 Jahre mit 4,4'-Diaminodiphenylsulfon.

A 3-year-old boy presented with a generalized bullous disease, clinically strongly indicative of chronic bullous disease of childhood (CBDC). The diagnosis was confirmed by histopathology and further verified by several immunological and biochemical examinations. Direct immunofluorescence (IF) of perilesional skin revealed in vivo bound IgA-autoantibodies (aabs) in a linear pattern along the basement membrane zone; indirect IF revealed circulating IgA-aabs bound to the roof of "split skin" preparations of healthy human skin; immunoblotting of epidermal protein extracts showed that the aabs bound to a 97KD/120KD protein. Therapy with 4,4'-diaminodiphenylsulfone (DADPS, 2 mg/kg /daily), combined with prednisolone for the first month, was initiated, and promptly led to complete remission. Two attempts to stop DADPS treatment after 3 and 5 years of continuous therapy were followed by prompt recurrences. The boy is now 8 years old; with continuous DADPS therapy (1 mg/kg body weight/d), he displays regular physical and intellectual development.

Narrow-band ultraviolet B (ATL-01) phototherapy is an effective and safe treatment option for patients with severe seborrhoeic dermatitis.

BACKGROUND: Seborrhoeic dermatitis is a common papulosquamous dermatosis affecting 2-10% of the adult population. Current treatment options are limited and not always satisfactory. Objectives We aimed to investigate the efficacy of narrow-band ultraviolet (UV) B (TL-01) phototherapy as an alternative treatment for seborrhoeic dermatitis. METHODS: Eighteen patients with severe disease were enrolled in an open prospective study. Treatment was given three times weekly until complete clearing or to a maximum of 8 weeks. A clinical score assessing erythema, scaling, infiltration and pruritus was performed at baseline and every 2 weeks thereafter. Additionally, the patients were asked to rate the intensity of pruritus on a visual analogue scale. After completion of the study the patients were followed up to determine the median time interval until recurrence. RESULTS: All patients responded favourably to treatment, with six showing complete clearance and 12 marked improvement. The median clinical score decreased from 7.5 (range 4-8) at baseline to 0.5 (range 0-3) after 8 weeks of treatment (P = 0.005). The median pruritus score decreased from 4.5 (range 0-8) at baseline to 0 (range 0-3) at week 8 (P = 0.008). Relapses occurred in all patients after a median of 21 days (range 12-40). No side-effects of treatment were observed except occasional episodes of a moderate erythemal response. CONCLUSIONS: Narrow-band UVB phototherapy appears to be a very effective and safe treatment option for patients with severe seborrhoeic dermatitis.

Immunotherapy of metastatic malignant melanoma by a vaccine consisting of autologous interleukin 2-transfected cancer cells: outcome of a phase I study.

We performed a phase I trial to evaluate the safety and tolerability of repeated skin injections of IL-2-transfected autologous melanoma cells into patients with advanced disease. Cell suspensions, propagated from excised metastases, were IL-2 gene transfected by adenovirus-enhanced transferrinfection and X-irradiated prior to injection. Vaccine production was successful in 54% of the patients. Fifteen patients (37%) received two to eight skin vaccinations of either 3 x 10(6) (intradermal) or 1 x 10(7) (half intradermal, half subcutaneous) transfected melanoma cells per vaccination (secreting 140-17,060 biological response modifier program units of IL-2/10(6) cells/24 hr). Analyses of safety and efficacy were carried out in 15 and 14 patients, respectively. Overall, the vaccine was well tolerated. All patients displayed modest local reactions (erythema, induration, and pruritus) and some experienced flu-like symptoms. Apart from newly appearing (4 of 14) and increasing (5 of 14) anti-adenovirus and newly detectable anti-nuclear antibody titers (1 of 15), recipients developed de novo or exhibited increased melanoma cell-specific delayed-type hypersensitivity (DTH) reactions (8 of 15) and vitiligo (3 of 15) and showed signs of tumor regression (3 of 15). This supports the idea of a vaccine-induced or -amplified anti-cancer immune response. None of the patients exhibited complete or partial regressions, but five of them experienced periods of disease stabilization. Three of these individuals received more than the four planned vaccinations and their mean survival time was 15.7 +/- 3.5 months as compared to 7.8 +/- 4.6 months for the entire patient cohort. These data indicate that IL-2-producing, autologous cancer cells can be safely administered to stage IV melanoma patients and could conceivably be of benefit to patients with less advanced disease.

[Established and new therapeutic approaches in dermatomyositis, polymyositis and overlapping syndromes]. / Etablierte und neue Therapiestrategien bei Dermatomyositis, Polymyositis und Uberlappungssyndromen.

An overview is presented on current antiinflammatory, cytostatic/cytotoxic and immunomodulatory treatments for immunologically mediated inflammatory myopathies with skin involvement. High-dose intravenous immunoglobulin (IVIG) therapy is discussed in the context of recent findings regarding pathogenic mechanisms of autoimmune diseases.

[Patch testing with the "Austrian standard series"--epidemiologic test values and results]. / Epikutantestung mit der "Standardreihe Osterreich"--Testepidemiologische Kenngrössen und Ergebnisse.

Patch testing for contact allergies is routinely performed by applying a "standard series" of the most frequently occurring contact allergens. Internationally "established" standard series have to be adapted to country- and population-specific factors (i.e., distribution of gender and age, occupational behavior, environmental factors etc.). In 1992 and 1993 altogether 11,690 patients were patch tested in 14 Austrian test centers with the "Austrian standard patch test series" as recommended by the working group "Contact Allergy" of the Austrian Society of Dermatology. Altogether data on 11,544 patients were complete and are analyzed in this study. 71.5% of the test population were female with an average age of 39 years, whereas the male patients had an average age of 40 years. 38.1% of the patients were younger than 30 years, 47.8% were in the group ranging from 31 to 60 years and 14% of the tested patients were older than 60 years. Cutaneous sensitization was found in 51% of the people tested with the "Austrian standard series". This proportion varied between 40.8 and 61.4% from center to center (with more than 100 tested persons). 15 (60%) of the 25 test substances in 1992 and 17 (68%) in 1993 surpassed the limit of a sensitization frequency of 1%. The "hit list" of substances did not differ qualitatively from those of other international studies, with only one remarkable, well described "Austrian-specific" exception, namely the mercury-containing thiomersal (second place).(ABSTRACT TRUNCATED AT 250 WORDS)

[Epidemiology and diagnosis of allergic diseases]. / Epidemiologie und Diagnose allergischer Erkrankungen.

Epidemiological studies indicate a world-wide and significant increase in atopic diseases over the past decades, which has adopted alarming dimensions within the industrialized world. However, allergic asthma and pollinosis, in particular, are on the increase in Third world countries, in parallel to the industrialization and westernization of their life-style. Since both antigen exposure and the presence of additional realization factors are required for the manifestation of atopic diseases, this increase in prevalence is not surprising. In addition to increased indoor and outdoor pollution, changes in the way of living--causing increased allergen exposure--certainly play an important role as cofactor in the increased incidence of allergies. Accurate diagnostic procedures permit a better understanding of the realization factors for allergic diseases in epidemiological studies and identification of the causative agent in the individual so that effective therapeutic and prophylactic steps can be taken. Despite improved in vivo and in vitro test methods, the case history is still of paramount importance in reaching a diagnosis; the potential hazards of skin and provocation testing, as well as the high costs and limitations of in vitro methods prohibit "blind allergy screening".