Unclassified vasculitis with acral ischemic lesions: "forme fruste" or idiopathic vasculitis?

While acral ischemia and necrosis represent a common problem in connective tissue diseases and other disorders, acral ischemic lesions are also occasionally encountered in primary and secondary systemic vasculitides. Here we report on the course of 4 patients with acral ischemic lesions as a hallmark of unclassified vasculitis. We compare these cases with 4 additional cases of acral ischemia complicating classified vasculitis.

To report on our experience with cases of unclassified vasculitis and acral ischemic lesions during the past 5 years and review the literature on vasculitis and acral ischemic lesions.

The case history of one of the patients with unclassified vasculitis and acral ischemic lesions is reported in detail. The Medical history of another 3 patients presenting with vasculitic acral ischemic lesions and unclassified vasculitis during the past 5 years in our department (Lübeck/Bad Bramstedt) is summarized and compared to the course of patients with acral ischemic lesions complicating classified vasculitides. A PubMed database review of reports on acral ischemic lesions and vasculitis from 1985 to August 2006 was performed using the following combination of keywords: "Vasculitis" [MeSH] AND ("Necrosis" [MeSH] OR "Ischemia" [MeSH] OR "Infarction" [MeSH]) AND ("Extremities" [MeSH] OR "Fingers [MeSH] OR "Toes" [MeSH] OR "limb"), yielding 1328 entries. This search was subsequently limited to "Humans, All Adult (19+ years)", yielding 904 entries. Only three (0.7%) of these entries described one (one paper) or more (n=28) patients (two papers) with idiopathic vasculitis characterized by digit necrosis in the absence of systemic manifestations (except in some cases for arthralgia) or laboratory parameters pointing to a diagnosis of an established type of vasculitis.

A 37-year-old female presented with acral ischemic lesions of the left forefoot, fingers and toes, and Raynaud's phenomenon. Angiography showed multiple stenoses of ulnar and digital arteries, anterior and posterior tibialis arteries, and occlusions of radial artery and occlusion of the plantar artery in the absence of large vessel abnormalities. Histological analysis of an amputation disclosed giant cell arteritis of small vessels. The patient achieved remission with immunosuppressive treatment (cyclophosphamide and prednisolone). Three other patients with acral ischemic lesions and unclassified vasculitis also lacking other manifestations and defining laboratory and technical features during initial presentation and follow-up of 4 month to 5 years are presented. Necrotizing and leukocytoclastic vasculitis were present in two other patients, respectively. In contrast, acral ischemic lesions could be attributed to rheumatoid vasculitis and essential cryoglobulinemic vasculitis in two other cases each based on the patient's history and laboratory findings at the time of presentation of acral ischemic lesions.

While acral ischemic lesions could represent initial or isolated (forme fruste) manifestations of a defined vasculitis, acral ischemic lesions may rarely be encountered as the predominant manifestation of an as yet unclassified vasculitis. the histological findings seem to differ. Our report includes a peculiar case of giant cell arteritis of small arteries not classifiable as giant cell arteritis of large arteries or Takayasu disease.