教育演講9:血管發炎的診療
Clinics in Vasculitis

E9-1
皮膚血管炎的分類及臨床表徵
Cutaneous vasculitis: classification and clinical manifestation
邱顯清
台大醫院皮膚科

  血管炎至今仍無一完善的分類法,不論以受侵犯血管的大小、浸潤的發炎細胞之種類、疾病的嚴重度(系統性或皮膚局限性)或病因之不同來分類,都有其不足之處。以下是皮膚血管炎分類的一種,雖然不盡完善,但包含了絕大多數常見的皮膚血管炎,臨床上具實用性。

Cutaneous Vasculitis
Predominantly small vessels
Leukocytoclastic vasculitis (LCV)
Henoch-Schonlein purpura
Urticarial vasculitis
Cryoglobulinemic vasculitis
Vasculitis associated with malignancy
Predominantly medium-sized vessels
Polyarteritis nodosa (classic and cutaneous)
Medium-sized and small vessels
ANCA-associated
Microscopic polyangitis
Wegener’s granulomatosis
Churg-Strauss syndrome
Drug-induced
Vasculitis associated with connective tissue diseases
(SLE, RA, Sjogren’s syndrome)
Other disorders that can show cutaneous LCV
Waldenstrom’s hypergammaglobulinemic purpura
Erythema elevatum diutinum
Granuloma faciale
Inflammatory bowel disease
Neutrophilic dermatoses (e.g., Behcet’s disease, pyoderma gangrenosum)
Arthropod bites
Nodular vasculitis

   皮膚血管炎中最常見的是 leukocytoclastic vasculitis,其臨床最大特徵是 palpable purpura;病理上則有四個主要變化:中性白血球浸潤、白血球細胞核裂解形成 nuclear dusts、小血管壁的 fibrinoid degeneration、以及紅血球的 extravasation。 LCV的病理機轉是含IgG的immune complex沈積於血管壁,誘發中性白血球浸潤導致血管炎。

  Henoch-Schonlein purpura 皮膚病灶的臨床及病理表現均與 LCV無法區別,但其 immune complex所含的抗體多為 IgA。

  Urticarial vasculitis的病灶與 urticaria相似,但個別病灶持續超過24小時,且病灶消失後常留下色素沈著或淤青,病理表現與LCV相同。