Differenzialdiagnose Angioödem

A woman presented in the emergency room with the diagnosis of angioedema refractory to treatment. She had soft, compressible periorbital edema, as well as edema of her hands and lower arms. She also complained of severe pain in her hands including sensations of numbness and tingling. The history, course and examination results eliminated several possible differential diagnostic considerations like an acute histamine- or bradykinin-mediated angioedema or superior vena cava syndrome. Histological examination confirmed the diagnosis of scleromyxedema.     

Omalizumab bei therapierefraktärer chronischer Urtikaria mit Angioödem

A 29-old man presented with severe chronic urticaria and angioedema. Routine evaluation including history, laboratory parameters and imaging procedures did not reveal any pathologic findings. We tried combined therapy regimes including high-dose systemic corticosteroids and antihistamines, leukotriene antagonist, cyclosporine and antibiotics. Because of a poor response and dramatic escalation of symptoms, we initiated therapy with omalizumab resulting in the complete remission of the chronic urticaria after two weeks.     

Differenzialdiagnostik von Lipödem und Lymphödem

Lipedema is a symmetrical disorder of the subcutaneous adipose tissue that affects almost exclusively women in postpubescent age. The trinity of disfiguring hyperplastic adipose tissue of the proximal extremities, increasing pain over time and bruising is characteristic. Lymphedema can occur in both sexes and is differentiated into primary and secondary subtypes. Symmetry is not a prerequisite for diagnosis. Characteristic for lymphedema is the disproportion between lymphatic fluid and the capacity of lymphatic vessels. The most distal body parts are always more severely affected than proximal parts. In the initial phases lymphedema is painless but in advanced stages tension pain can occur. The combination of both disorders has been described; however, lipedema is not responsible for subsequent lymphedema in contrast to central obesity, which significantly increases the risk of lymphedema. The differential diagnosis is of utmost importance for a meaningful management.     

Skleromyxödem

Ohne ZusammenfassungMit 12 Textabbildungen. Georg Arndt zum Gedächtnis anläßlich seines 80. Geburtstages (geb. am 2. 8. 1874) und seines 25 jährigen Todestages (gest. am 19. 8. 1929).     

Skleromyxödem

Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy. The pathogenesis of scleromyxedema is unknown. The clinical course is chronic and progressive and can lead to marked morbidity or death. The skin findings consist of multiple waxy papules and indurated plaques. Progressive skin involvement can lead to decreased mobility of the mouth and joints. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, or in the kidneys. There are no approved or evidence-based treatment options available for scleromyxedema. High-dose immunoglobulins are considered the treatment of choice, followed by lenalidomide (or thalidomide) and systemic glucocorticosteroids, or in severe cases even autologous hematopoetic stem cell transplantation. Long-term maintenance treatment is usually required and close clinical follow-up is necessary as recurrence of scleromyxedema is common after withdrawal of an effective therapy.     

Kälteinduzierte Quaddeln und Angioödeme

The onset of wheals and/or angioedema following the exposure to cold may be associated with a number of different diseases. Most frequently this occurs in cold contact urticaria, a type of physical urticaria, which is characterized by a positive cold stimulation test. The clinical symptoms are based on cold-dependent mast cell activation with subsequent release of proinflammatory mediators. In cases of negative or atypical reaction to cold stimulation testing rare acquired atypical or familiar cold urticaria forms may be suspected. Strict avoidance of cold should be recommended as far as possible. As the underlying causes of cold contact urticaria are widely unknown, the symptomatic use of non-sedating antihistamines is the treatment of first choice. The very rare familiar cold auto-inflammatory syndrome (FCAS) is based on CIAS1/NLRP3 mutations and may be treated effectively by neutralization of pathogenic interleukin 1β.     

Angioödeme durch ACE-Hemmer und AT1-Rezeptorblocker

Acute drug-induced angioedema is a non-pruritic swelling of subcutaneous and submucosal tissue. It most occur occurs the mouth and larynx as well as lips and eyelids; less often other skin regions or even the gut can be involved. ACE inhibitor (ACEi)- induced angioedema typical occurs after several years on medication. A 72-year-old woman developed recurrent ACEi-induced angioedema. The drug was stopped and she received an AT(1)-receptor antagonist as an alternative antihypertensive. This medication also led to angioedema, which was not immediately recognized as medication-associated. AT(1)-receptor antagonists are not suitable alternatives for ACEi because they also carry an increased risk for inducing angioedema.     

Medikamente als Auslöser Bradykinin-vermittelter Angioödeme – mehr als ACE-Hemmer

Bradykinin-mediated angioedema is a rare, non-allergic, potentially life-threatening disease. ACE inhibitor-induced angioedema and hereditary angioedema (HAE) are the two most common presentations. Therapeutic options, pathophysiology and diagnosis continue to be investigated, with considerable progress in HAE over the last few decades. For all patients with bradykinin-mediated angioedema, there are several medications that should be avoided or administered with caution. Some of the triggering medications are well known, while others are suspected or of unknown significance. A common denominator is that there is no approved therapy for bradykinin-mediated angioedema as a drug side effect. Some medications, such as tissue plasminogen activator, have a higher incidence of angioedema with potential airway compromise than ACE inhibitors, although this fact is widely underappreciated. In this review, we aim to summarize what is currently known and recommended about concomitant medication in HAE patients and the interaction of other bradykinin-influencing drugs.     

Über gonorrhoisches Skrotalödem

Ohne Zusammenfassung     

Skleromyxödem (Arndt-Gottron)

Zusammenfassung Das zu den Mucinosen der Haut gehörende Skleromyxödem wurde infolge seiner großen Seltenheit erst einige Male beschrieben.Ein ausgeprägter Fall eines Skleromyxödems bei einer 57 jährigen Patientin kam an unserer Klinik zur Beobachtung. Die klinischen Symptome bestanden in einer generalisierten Aussaat von hirsckorngroßen, weißlich-gelben, harten Knötchen, die an einzelnen Stellen auch zu parallel verlaufenden Ketten aneinandergereiht waren. Weiters bestand eine imponierende, ausgedehnte Verhärtung und Verdickung der Haut, die über den großen Gelenken eine eigenartige, grobwulstige Faltenbildung verursachte. Im Bereich der Cubitalgelenke, in geringerem Ausmaße auch in den Kniegelenken, lag eine merkliche Bewegungseinschränkung vor. Durch die histologische Untersuchung konnte in der Cutis eine diffuse Einlagerung eines Stoffes festgestellt werden, dessen Natur durch die histochemischen und histoenzymatischen Untersuchungen als Schleim definiert werden konnte.Obwohl über die Pathogenese der Erkrankung in letzter Zeit verschiedene Anhaltspunkte gewonnen wurden, ist dieselbe noch keineswegs geklärt. Die klinische und histologische Ähnlichkeit der Erkrankung mit anderen Mucinosen der Haut lassen vermuten, daß endokrine Faktoren im Spiele sind. Auf die Rolle lokaler degenerative Veränderungen des Bindegewebes, das vielfach eine Umwandlung in Schleimsubstanz erfährt, wird hingewiesen. Dieser zwar nur sekundär in Erscheinung tretende Faktor ist sicherlich im Prozeß der Schleimeinlagerung in der Cutis von wesentlicher Bedeutung.Mit 12 Textabbildungen     

Ein Fall von atypischem tuberösem Myxödem

Ohne ZusammenfassungMit 3 Textabbildungen.Die Moulage und die mikroskopischen Präparate des Falles wurden von mir im Jahre 1920 in der Moskauer Venerologischen Gesellschaft demonstriert, als mir der FallDösseckers noch unbekannt war.     

Pseudomonas-aeruginosa-Infektion unter dem Bild einer Gonorrhö

A 68 year old man presented with urethritis and a purulent discharge, carrying the tentative diagnosis of gonorrhea. He had already been treated with multiple antibiotics. Microbiological investigation revealed Pseudomonas aeruginosa, a relatively frequent Gram-negative bacteria in hospitals, which can cause several nosocomial diseases such as pneumonia, wound infections and urogenital infections. Therapy can be difficult because of frequent antibiotic resistance. Guided by sensitivity studies, the patient was successfully treated with gyrase inhibitors. Pseudomonas aeruginosa-induced urogenital infections in ambulatory patients are extremely rare and usually not associated with a gonorrhea-like discharge.     

Fall 14. Skleromyxödem Arndt-Gottron

Ohne Zusammenfassung