Zusammenfassung
Akrale Durchblutungsstörungen zeichnen sich durch wenige, uniforme klinische Erscheinungen und eine Vielzahl möglicher Pathomechanismen aus. Der vorliegende Beitrag konzentriert sich auf die funktionellen Formen. Dem Raynaud-Phänomen liegen kälte- oder stressinduzierte Vasospasmen kleiner akraler Arterien zugrunde. Es ist definiert über die Farbveränderungen der Haut, im typischen Fall weiß-blau-rot (Trikolore). Am häufigsten betroffen sind die Langfinger. Die Ätiologie ist unbekannt, die Pathophysiologie nur unzureichend verstanden. Es werden ein primäres und ein sekundäres Raynaud-Phänomen unterschieden. Wichtigste Grunderkrankungen sind Kollagenosen, vorrangig die systemische Sklerose, und Malignome, ferner können Medikamente und Drogen Vasospasmen induzieren. Die Therapie zielt auf die Verhütung oder Durchbrechung der Vasospasmen, ist dabei aber bisher nur bedingt wirksam. Die Akrozyanose ist eine vasospastisch-dystone akrale Erkrankung, die zu einer permanenten rötlich-lividen Verfärbung vor allem der Hände und Füße führt. Sekundäre Formen kommen bei Kollagenosen, Malignomen und myelodysplastischen Syndromen vor. Ätiologie und Pathophysiologie sind weitgehend unbekannt. Eine wirksame pharmakologische Beeinflussung ist nicht möglich. Im Gegensatz zu allen anderen vaskulären Akrosyndromen zeichnet sich die Erythromelalgie durch eine Hyperämie aus. Die primäre Form ist eine genetisch bedingte Natriumkanalerkrankung. Sekundäre Formen kommen bei Malignomen, Kollagenosen und myelodysplastischen Syndromen vor. Die Beschwerden sind oft quälend und invalidisierend. Die Therapie erfordert einen multimodalen Ansatz, der nichtpharmakologische und pharmakologische Strategien umfasst. Eine enge interdisziplinäre Zusammenarbeit ist im Management dieser Erkrankung unabdingbar.
Abstract
Vascular acrosyndromes are characterized by sparse, uniform clinical manifestations and a variety of possible pathomechanisms. The present article focuses on the functional entities. Raynaud phenomenon is based on cold- or stress-induced vasospasms of acral arteries. It is defined by the color changes of the skin, in the typical case white–blue–red (tricolore). The long fingers are most commonly affected. The etiology is unknown, and the pathophysiology is poorly understood. A distinction is made between primary and a secondary Raynaud phenomenon. The most important underlying diseases include collagenosis, primarily systemic sclerosis, and malignancies; furthermore, medications and drugs may promote vasospasm. Treatment is aimed at preventing or breaking the vasospasm, but has been only partially effective in doing so. Acrocyanosis is a vasospastic dystonic acral disorder that results in permanent reddish-livid discoloration, especially of the hands and feet. Secondary forms occur in collagenosis, malignancies, and myelodysplastic syndromes. The etiology and pathophysiology are virtually unknown. Targeted pharmacological intervention is not possible. Unlike all other vascular acrosyndromes, erythromelalgia is characterized by hyperemia. The primary form is a genetic sodium channelopathy, while secondary forms include malignancies, connective tissue diseases, and myelodysplastic syndromes. The symptoms are often distressing and disabling. Therapy requires a multimodal approach that includes both nonpharmacological and pharmacological strategies. Close interdisciplinary collaboration is essential for the management of this disease.
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P. Klein-Weigel, A. Ruttloff, D. König, J. Nielitz, J. Steindl, O. Sander und J.G. Richter geben an, dass kein Interessenkonflikt besteht.
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Klein-Weigel, P., Ruttloff, A., König, D. et al. Funktionelle akrale Durchblutungsstörungen. Internist 63, 591–600 (2022). https://doi.org/10.1007/s00108-022-01340-w
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DOI: https://doi.org/10.1007/s00108-022-01340-w