Kutane Sarkoidose

Zusammenfassung

Die Sarkoidose ist eine Multisystemerkrankung, deren Ursache bislang ungeklärt ist. Pathognomonisch sind nicht verkäsende Granulome, die alle Gewebe oder Organsysteme betreffen können. Aufgrund der guten klinischen Sichtbarkeit der kutanen Sarkoidose ist diese oft der erste Hinweis für das Vorliegen einer systemischen Sarkoidose. Die Dermatologie spielt somit eine wichtige Rolle bei der Früherkennung dieser multisystemischen Erkrankung. Eine reine Hautmanifestation ohne systemische Beteiligung ist möglich. Die häufigsten Vertreter hierfür sind die makulopapulöse und die noduläre Sarkoidose. Zum anderen gibt es unspezifische Hautmanifestationen, wie z.B. das Erythema nodosum, welches im Rahmen einer systemischen Sarkoidose auftreten kann. Die Diagnose wird mittels Gewebebiopsie gestellt. Die Biomarker C-reaktives Protein (CRP), löslicher Interleukin-2 Rezeptor (sIl-2 R), ACE und ggf. Neopterin können bei Hautmanifestation Hinweis für eine systemische Beteiligung sein. Die Therapie richtet sich nach Schwere der Hautmanifestation und systemischer Beteiligung. Zur Behandlung der kutanen Sarkoidose zugelassen sind topische, intraläsionale oder systemische Glukokortikosteroide. Bei therapierefraktären Verläufen können off-label immunmodulierende oder immunsuppressive Medikamente eingesetzt werden.

Abstract

Sarcoidosis is a systemic disease whose etiology is still undetermined. It is characterized by the presence of non-caseating granulomas that can affect all tissues or organ systems. Cutaneous sarcoidosis has a pronounced clinical visibility which often serves as the first indication of systemic sarcoidosis. Thus, dermatology plays a decisive role in the early detection of this multisystemic disease. However, sarcoidosis can have isolated manifestations without systemic involvement, with maculopapular and nodular sarcoidosis as the most common examples. Additionally, there are non-specific skin manifestations such as erythema nodosum, which can occur in conjunction with systemic sarcoidosis. Tissue biopsy is the main procedure for confirming the diagnosis. In cases with skin manifestations, biomarkers such as CRP, sIL-2R and neopter may indicate systemic sarcoidosis. The severity of the skin manifestation and systemic involvement determine the therapy to be used. Topical, intralesional or systemic glucocorticosteroids are approved for the treatment of cutaneous sarcoidosis. Off-label immunomodulatory or immunosuppressive drugs can be used for refractory cases.

Fazit

• Die Sarkoidose der Haut kann isoliert auftreten und einen selbstlimitierenden Verlauf aufzeigen.

• Eine kutane Beteiligung kann Erstmanifestation einer systemischen Sarkoidose sein.

• Lösliches IL-2-Rezeptor, Neopterin und C-reaktives Protein sind wegweisend für eine Systembeteiligung.

• Die Diagnosesicherung erfolgt mittels Gewebeprobe.

• Die Therapieentscheidung ist abhängig von der klinischen Ausprägung und extrakutanen Beteiligung.

• Glukokortikosteroide sind die einzig zugelassene Therapie.

• Immunmodulatoren oder Immunsuppressiva sind Off-Label-Therapieoptionen.

Schlüsselwörter

Kutane Sarkoidose - Erythema nodosum - Lupus pernio - Darier-Roussy-Sarkoidose - makulopapulöse Sarkoidose

Keywords

Cutaneous sarcoidosis - erythema nodosum - lupus pernio - Darier-Roussy sarcoidosis - maculopapular sarcoidosis

Publication History

Article published online:
12 March 2025

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