E. Ardelean

Anti-Immunoglobulin E Treatment of Patients with Recalcitrant Physical Urticaria

In physical urticaria, exogenous physical factors such as thermal triggers, solar radiation and mechanic triggers including friction or pressure are responsible for the elicitation of symptoms in the skin of patients. Avoidance of the respective stimulus is usually difficult or impossible, and many patients are not sufficiently treated with standard antihistamines. We report that treatment with omalizumab (Xolair®) of 7 patients with physical urticarias [solar urticaria (n = 2), urticaria factitia/symptomatic dermographism (n = 2), cold urticaria, delayed pressure urticaria and localized heat urticaria] resulted in complete symptom control within days after the first injection in 5 patients. In 1 patient, symptoms improved after increasing the dose of omalizumab, and 1 patient with localized heat urticaria did not respond significantly to treatment. Before anti-immunoglobulin E treatment, all patients had suffered from their physical urticaria for years and had had numerous unsuccessful therapies. The overall excellent responses to omalizumab treatment reported here indicate that anti-immunoglobulin E is a safe and effective treatment for recalcitrant physical urticarias.

Selected urticaria patients benefit from a referral to tertiary care centres – results of an expert survey

Background  Urticaria is a frequent reason for consultations. Recently, it has been demonstrated that the management of chronic spontaneous urticaria (csU) in the practice setting does not fully comply with published guidelines. In addition, it was shown that one of four csU patients is referred to specialized centres.

[Chronic urticaria. Prevalence, course, prognostic factors and impact].

Chronic urticaria (CU) is one of the most frequent diseases in the field of dermatology. Recent studies have shown a point prevalence between 0.5 and 1% in the total population with a predominance of females. In general, all age groups and all classes of the population can be affected. An incidence peak has been found in the third and fourth decades. According to the current guidelines, CU is characterized by the spontaneous occurrence of wheals and/or angioedema for more than 6 weeks. However, epidemiological studies have revealed that the majority of patients suffer for several months, or frequently years. Disease duration is likely to be longer in case of angioedema, a combination with physical urticaria, positivity in the autologous serum skin test (autoreactivity) and a high disease severity. Studies on the impairment of quality of life have been shown that many CU patients suffer as strong from their disease as patients with coronary artery disease. Apart from pure physical symptoms, patients experience restrictions in daily life activities and social life. In addition, sleep disturbances are common and CU patients frequently exhibit psychiatric comorbidities. To avoid frustration in care, it is important to perceive all different dimensions of CU that impact the patients life and to take the patients and their disease seriously. The aim of therapy should be to obtain total symptom control.

[Cold-induced urticaria and angioedema. Classification, diagnosis and therapy].

ZusammenfassungDem Auftreten von Quaddeln und/oder Angioödemen bei Kälte können verschiedene Erkrankungen zugrunde liegen. Am häufigsten ist dies die Kältekontakturtikaria, eine physikalische Urtikariaform, die durch einen positiven Kältestimulationstest definiert ist. Den klinischen Symptomen liegt eine kälteabhängige Mastzellaktivierung mit anschließender Freisetzung proinflammatorischer Mediatoren zugrunde. Bei fehlender oder atypischer Reaktion im Kältestimulationstest sind differenzialdiagnostisch die weitaus seltener vorkommenden erworbenen atypischen und familiären Kälteurtikariaformen zu erwägen. Therapeutisch steht das konsequente Meiden des Triggerfaktors Kälte an erster Stelle. Da die zugrunde liegenden Ursachen der Kältekontakturtikaria weitgehend unbekannt sind, basiert die medikamentöse Behandlung hauptsächlich auf dem symptomatischen Einsatz nicht sedierender Antihistaminika. Das äußerst seltene familiäre kälteinduzierte autoinflammatorische Syndrom (FCAS), das auf CIAS1/NLRP3-Mutationen beruht, spricht therapeutisch gut auf eine Neutralisation des pathogen wirkenden Interleukin-1β an.AbstractThe 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.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 1beta.

Kälteinduzierte Quaddeln und Angioödeme

ZusammenfassungDem Auftreten von Quaddeln und/oder Angioödemen bei Kälte können verschiedene Erkrankungen zugrunde liegen. Am häufigsten ist dies die Kältekontakturtikaria, eine physikalische Urtikariaform, die durch einen positiven Kältestimulationstest definiert ist. Den klinischen Symptomen liegt eine kälteabhängige Mastzellaktivierung mit anschließender Freisetzung proinflammatorischer Mediatoren zugrunde. Bei fehlender oder atypischer Reaktion im Kältestimulationstest sind differenzialdiagnostisch die weitaus seltener vorkommenden erworbenen atypischen und familiären Kälteurtikariaformen zu erwägen. Therapeutisch steht das konsequente Meiden des Triggerfaktors Kälte an erster Stelle. Da die zugrunde liegenden Ursachen der Kältekontakturtikaria weitgehend unbekannt sind, basiert die medikamentöse Behandlung hauptsächlich auf dem symptomatischen Einsatz nicht sedierender Antihistaminika. Das äußerst seltene familiäre kälteinduzierte autoinflammatorische Syndrom (FCAS), das auf CIAS1/NLRP3-Mutationen beruht, spricht therapeutisch gut auf eine Neutralisation des pathogen wirkenden Interleukin-1β an.AbstractThe 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.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 1beta.

Kälteinduzierte Quaddeln und Angioödeme@@@Cold-induced urticaria and angioedema: Klassifikation, Diagnostik und Therapie@@@Classification, diagnosis and therapy

ZusammenfassungDem Auftreten von Quaddeln und/oder Angioödemen bei Kälte können verschiedene Erkrankungen zugrunde liegen. Am häufigsten ist dies die Kältekontakturtikaria, eine physikalische Urtikariaform, die durch einen positiven Kältestimulationstest definiert ist. Den klinischen Symptomen liegt eine kälteabhängige Mastzellaktivierung mit anschließender Freisetzung proinflammatorischer Mediatoren zugrunde. Bei fehlender oder atypischer Reaktion im Kältestimulationstest sind differenzialdiagnostisch die weitaus seltener vorkommenden erworbenen atypischen und familiären Kälteurtikariaformen zu erwägen. Therapeutisch steht das konsequente Meiden des Triggerfaktors Kälte an erster Stelle. Da die zugrunde liegenden Ursachen der Kältekontakturtikaria weitgehend unbekannt sind, basiert die medikamentöse Behandlung hauptsächlich auf dem symptomatischen Einsatz nicht sedierender Antihistaminika. Das äußerst seltene familiäre kälteinduzierte autoinflammatorische Syndrom (FCAS), das auf CIAS1/NLRP3-Mutationen beruht, spricht therapeutisch gut auf eine Neutralisation des pathogen wirkenden Interleukin-1β an.AbstractThe 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.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 1beta.