K. Gensch

Photoprotective effects of a broad-spectrum sunscreen in ultraviolet-induced cutaneous lupus erythematosus: A randomized, vehicle-controlled, double-blind study

OBJECTIVE We sought to assess if the exclusive use of a broad-spectrum sunscreen can prevent skin lesions in patients with different subtypes of cutaneous lupus erythematosus (CLE) induced by ultraviolet (UV) irradiation under standardized conditions. METHODS A total of 25 patients with a medical history of photosensitive CLE were included in this monocentric, randomized, vehicle-controlled, double-blind, intraindividual study. The test product and its vehicle were applied 15 minutes before UVA and UVB irradiation of uninvolved skin areas on the upper aspect of the back in a random order, and standardized phototesting was performed daily for 3 consecutive days. RESULTS Characteristic skin lesions were induced by UVA and UVB irradiation in 16 patients with CLE in the untreated area, and 14 patients showed a positive test result in the vehicle-treated area. In contrast, no eruptions compatible with CLE were observed in the sunscreen-treated area in any of the 25 patients. This resulted in significant differences (P < .001) between UV-irradiated sunscreen-treated versus vehicle-treated areas, and between UV-irradiated sunscreen-treated versus untreated areas. Furthermore, a significant difference (P < .05) was observed concerning the age of disease onset and the patient history of photosensitivity. Patients who were younger than 40 years at onset of CLE reported photosensitivity significantly more often than patients with a higher age of disease onset. None of the patients showed any adverse events from application of the test product or the vehicle. LIMITATIONS Data resulting from standardized experimental phototesting might not be transferable to a clinical setting. CONCLUSION These results indicate clearly that the use of a highly protective broad-spectrum sunscreen can prevent skin lesions in photosensitive patients with different subtypes of CLE.

Connective tissue diseases in childhood

ZusammenfassungKollagenosen umfassen eine heterogene Gruppe von chronisch entzündlichen Multiorganerkrankungen, die den systemischen Lupus erythematodes (SLE), die progressiv systemische Sklerodermie (PSS), die Dermato- (DM) und Polymyositits (PM) sowie die gemischte Bindegewebserkrankung („mixed connective tissue disease“, MCTD) und das Sjögren-Syndrom (SS) einschließen. Insbesondere zu Beginn der jeweiligen Erkrankung können die Patienten ähnliche klinische Symptome aufweisen, die häufig eine spezifische Diagnose erschweren. Im Kindesalter ist die Inzidenz der Kollagenosen wesentlich niedriger als bei Erwachsenen, aber die Krankheitsbilder zeigen eine größere morphologische Vielfältigkeit. Allgemeinsymptome wie Müdigkeit, Fieber und Gewichtsverlust können einer systemischen Organmanifestation vorausgehen, und während des rezidivierenden Verlaufs der Erkrankungen entwickeln sich bei Kindern am häufigsten mukokutane Manifestationen. In diesem Übersichtsartikel werden die neuesten Angaben über Epidemiologie, klinische Manifestationen, diagnostisches Prozedere und therapeutische Strategien der Kollagenosen zusammengefasst und spezifische Probleme im Kindesalter dargestellt.AbstractConnective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögren’s syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.Connective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögrens syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.

Kollagenosen im Kindesalter

ZusammenfassungKollagenosen umfassen eine heterogene Gruppe von chronisch entzündlichen Multiorganerkrankungen, die den systemischen Lupus erythematodes (SLE), die progressiv systemische Sklerodermie (PSS), die Dermato- (DM) und Polymyositits (PM) sowie die gemischte Bindegewebserkrankung („mixed connective tissue disease“, MCTD) und das Sjögren-Syndrom (SS) einschließen. Insbesondere zu Beginn der jeweiligen Erkrankung können die Patienten ähnliche klinische Symptome aufweisen, die häufig eine spezifische Diagnose erschweren. Im Kindesalter ist die Inzidenz der Kollagenosen wesentlich niedriger als bei Erwachsenen, aber die Krankheitsbilder zeigen eine größere morphologische Vielfältigkeit. Allgemeinsymptome wie Müdigkeit, Fieber und Gewichtsverlust können einer systemischen Organmanifestation vorausgehen, und während des rezidivierenden Verlaufs der Erkrankungen entwickeln sich bei Kindern am häufigsten mukokutane Manifestationen. In diesem Übersichtsartikel werden die neuesten Angaben über Epidemiologie, klinische Manifestationen, diagnostisches Prozedere und therapeutische Strategien der Kollagenosen zusammengefasst und spezifische Probleme im Kindesalter dargestellt.AbstractConnective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögren’s syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.Connective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögrens syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.

New Trends in Topical and Systemic Immunosuppressive Treatment

Publisher Summary This chapter discusses new trends in topical and systemic immunosuppressive treatment. The substances discussed in the chapter are examples of new topical and systemic therapeutic options in the therapy of systemic lupus erythematosus (SLE) and other systemic autoimmune diseases, which indicate the enormous development in this area. However, one important future step is the initiation of optimized clinical trials, which need to take into account the heterogeneous expression of SLE and the lack of proven standardized therapy for specific organ manifestations, such as the central nervous system (CNS) and skin. The American College of Rheumatism (ACR) has developed standardized response criteria to measure the overall disease activity in SLE providing a common basis for comparing treatment options, permitting more diverse patients to be studied and to be compared statistically, and facilitating qualitative and quantitative (meta-analysis) synthesis of different clinical trials.

Kollagenosen im Kindesalter@@@Connective tissue diseases in childhood

ZusammenfassungKollagenosen umfassen eine heterogene Gruppe von chronisch entzündlichen Multiorganerkrankungen, die den systemischen Lupus erythematodes (SLE), die progressiv systemische Sklerodermie (PSS), die Dermato- (DM) und Polymyositits (PM) sowie die gemischte Bindegewebserkrankung („mixed connective tissue disease“, MCTD) und das Sjögren-Syndrom (SS) einschließen. Insbesondere zu Beginn der jeweiligen Erkrankung können die Patienten ähnliche klinische Symptome aufweisen, die häufig eine spezifische Diagnose erschweren. Im Kindesalter ist die Inzidenz der Kollagenosen wesentlich niedriger als bei Erwachsenen, aber die Krankheitsbilder zeigen eine größere morphologische Vielfältigkeit. Allgemeinsymptome wie Müdigkeit, Fieber und Gewichtsverlust können einer systemischen Organmanifestation vorausgehen, und während des rezidivierenden Verlaufs der Erkrankungen entwickeln sich bei Kindern am häufigsten mukokutane Manifestationen. In diesem Übersichtsartikel werden die neuesten Angaben über Epidemiologie, klinische Manifestationen, diagnostisches Prozedere und therapeutische Strategien der Kollagenosen zusammengefasst und spezifische Probleme im Kindesalter dargestellt.AbstractConnective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögren’s syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.Connective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögrens syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.