Diagnostics of autoimmune bullous diseases in German dermatology departments

Summary Background: No consistent data are available on the currently employed diagnostic tools for autoimmune bullous diseases in Germany. The aim of this survey was to describe currently performed diagnostic methods for bullous autoimmune diseases in German dermatology departments. Methods: A standardized questionnaire evaluated the available diagnostic methods i. e. direct immunofluorescence microscopy (IFM), indirect IFM, commercial ELISA systems, and non-commercial serological tests as well as the number of samples per year in all 34 university and 39 non-university dermatology departments. Results: The overall return rate was 89 %, 100 % and 79 % for the university and non-university …

Granulomatous mycosis fungoides, a rare subtype of cutaneous T-cell lymphoma

Granulomatous mycosis fungoides (GMF) is an unusual histologic subtype of cutaneous T-cell lymphoma.1 The diagnosis of GMF is usually established after observation of a granulomatous inflammatory reaction associated with a malignant lymphoid infiltrate. Epidermotropism, a clue to diagnosis in classical mycosis fungoides (MF) may be absent in about 47% of cases of GMF.2 In some instances, the granulomatous component may be intense and obscures the lymphomatous component of the infiltrate.1 There are no distinctive clinical patterns associated with GMF.1, 3

Regulatory T cell deficient scurfy mice exhibit a Th2/M2-like inflammatory response in the skin

Abstract Background Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). Objective Autoimmune skin inflammation and ANA are hallmarks for the diagnosis of autoimmune connective tissue diseases; therefore we analyzed scurfy mice for typical signs of these diseases. Methods Indirect immunofluorescence was used to specify the ANA pattern in scurfy mice.…

Diagnostik blasenbildender Autoimmundermatosen an deutschen Hautkliniken

European Guidelines (S1) on the use of high‐dose intravenous immunoglobulin in dermatology

Background The treatment of severe dermatological autoimmune diseases and toxic epidermal necrolysis (TEN) with high-dose intravenous immunoglobulin (IVIg) is a well-established procedure in dermatology. As treatment with IVIg is usually considered for rare clinical entities or severe clinical cases, the use of immunoglobulin is not generally based on data from randomized controlled trials that are usually required for the practice of evidence-based medicine. Owing to the rarity of the indications for the use of IVIg, it is also unlikely that such studies will be available in the foreseeable future. Because the high costs of IVIg treatment also limit its first-line use, the first clinical g…

Europäische Leitlinien (S1) für die Anwendung von hochdosierten intravenösen Immunglobulinen in der Dermatologie.

Hintergrund und Ziele Die Behandlung schwerer dermatologischer Autoimmunerkrankungen und der toxischen epidermalen Nekrolyse (TEN) mit hochdosierten intravenosen Immunglobulinen (IVIg) ist ein bewahrtes therapeutisches Verfahren in der Dermatologie. Da eine IVIg‐Therapie in der Regel nur bei seltenen Erkrankungen oder bei schweren Fallen in Betracht gezogen wird, stutzt sich die Anwendung von Immunglobulinen zumeist nicht auf Daten aus randomisierten kontrollierten Studien, wie sie in der evidenzbasierten Medizin erforderlich sind. Da Indikationen fur die Anwendung von IVIg selten sind, ist es unwahrscheinlich, dass solche Studien in absehbarer Zeit durchgefuhrt werden. Wegen der hohen Kost…