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Kutane Vaskulitis und Vaskulopathie

Differenzialdiagnosen an der unteren Extremität

Cutaneous vasculitis and vasculopathy

Differential diagnosis in biopsies of the lower extremities

Zusammenfassung

Die Haut zählt zu den häufigsten Manifestationsorten systemischer primärer und sekundärer Vaskulitiden. Ferner tritt eine Vaskulitis nicht selten rein kutan als Einzelorganvaskulitis auf. Prädilektionsorte sind die unteren Extremitäten mit den Leitsymptomen palpable Purpura, Knoten und Ulzera. Grundlage der histopathologischen Diagnostik ist die korrekte Einordnung des befallenen Gefäßtyps und die sichere Identifizierung einer Gefäßschädigung bei der Vaskulitis in Abgrenzung zu unspezifischen reaktiven Gefäßveränderungen, vaskulär okklusiven Erkrankungen, nichtinflammatorischer Purpura und perivaskulären Infiltraten infolge entzündlicher Dermatosen. Die Mehrzahl kutaner Vaskulitiden sind Kleingefäßvaskulitiden. Diese weisen im floriden Stadium unabhängig von der Ätiologie meist das Bild einer dermalen leukozytoklastischen Vaskulitis auf. Zusätzliche extravaskuläre Veränderungen können eine weitere Einordnung erlauben, in der Regel ist jedoch eine Korrelation mit Klinik, Bildgebung und Serologie erforderlich. Entscheidend für die Diagnose ist auch die Entnahmetechnik der Biopsie unter Berücksichtigung des klinisch vermuteten Gefäßbefalls. Gerade bei Ulzera ist ein ausreichender Saum umgebender Haut und Subkutis diagnostisch unerlässlich. Für die Diagnose einer Polyarteriitis nodosa und nodulärer Vaskulitis ist die Beurteilung der Subkutis erforderlich. Eine Großgefäßvaskulitis kommt in Hautbiopsien der unteren Extremität nicht vor.

Abstract

The skin is one of the most frequently involved organs in primary systemic and secondary vasculitis; moreover, a vasculitis can occur as single organ vasculitis, limited to the skin. For most types of vasculitis, the lower extremities constitute common sites with clinical symptoms of palpable purpura, nodules, and ulcers. In histopathology of cutaneous vasculitis, it is of utmost importance to correctly identify the vessel types involved and to discriminate between vasculitic vessel damage, unspecific reactive vessel changes, vascular occlusive diseases, noninflammatory purpura, or perivascular infiltrates due to other inflammatory skin diseases. Small-vessel vasculitis is the most frequent type of cutaneous vasculitis; during florid phases, a dermal leukocytoclastic vasculitis is found regardless of etiology. Additional extravascular changes may give etiological clues, however, a correlation with the clinical picture, radiology, and serology is essential. The biopsy type and technique need to be adjusted to the suspected diagnosis and site of the vessels involved. Polyarteritis nodosa and nodular vasculitis can be diagnosed only in biopsies with sufficient subcutaneous tissue. Especially in cutaneous ulcers, a lateral rim of vital skin and subcutaneous tissue is indispensable for a correct diagnosis. Large-vessel vasculitis is not found in skin biopsies of the lower extremities.

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Correspondence to K. Holl-Ulrich.

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K. Holl-Ulrich und C. Rose geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Holl-Ulrich, K., Rose, C. Kutane Vaskulitis und Vaskulopathie. Pathologe 41, 355–363 (2020). https://doi.org/10.1007/s00292-020-00786-9

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  • DOI: https://doi.org/10.1007/s00292-020-00786-9

Schlüsselwörter

  • Leukozytoklastische Vaskulitis
  • ANCA
  • Polyarteriitis nodosa
  • Noduläre Vaskulitis
  • Okklusive Vaskulopathie

Keywords

  • Leukocytoclastic vasculitis
  • ANCA
  • Polyarteritis nodosa
  • Nodular vasculitis
  • Occlusive vasculopathy