The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

Kraemer, Markus; Linden, Dieter; Berlit, Peter

doi:10.1007/s00415-005-0967-9

Cited by 77 publications

(75 citation statements)

References 90 publications

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“…Livedo was noted before cerebrovascular events in more than half of the patients with Sneddon syndrome in two reported studies. 20,23 In our series, a statistically significant association between histopathological necrotizing vasculitis in the skin biopsy specimen and ischemic cerebrovascular events was found among patients in whom livedo was noted. These findings indicate that histopathological cutaneous necrotizing vasculitis may be a useful parameter to define the risk of ischemic cerebrovascular events.…”

Section: Livedo Racemosasupporting

confidence: 48%

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

Kawakami

2010

The Journal of Dermatology

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Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg-Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch-Schö nlein purpura is indicated. Finally, the presence of anti-phosphatidylserine-prothrombin complex antibodies and ⁄ or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and ⁄ or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch-Schö nlein purpura.

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Section: Livedo Racemosasupporting

confidence: 48%

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

Kawakami

2010

The Journal of Dermatology

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“…There are no speciWc criteria for diagnosis of SS; therefore, the clinical observation of livedo reticularis and neuroradiological conWrmation of [4,12], are key factors in the diagnosis of SS [11]. In this study, arterial hypertension was the only risk factor for cerebrovascular disease found to be more prevalent in patients with PAPS with SS than in those with PAPS without SS, in agreement with observations made in previous studies [4,11].…”

Section: Discussionsupporting

confidence: 94%

“…The cause of SS is still unclear, its aetiopathology remains controversial, and its association with antiphospholipid antibodies is under debate [3,12].…”

Section: Discussionmentioning

confidence: 99%

Primary antiphospholipid syndrome with and without Sneddon’s syndrome

Caldas

Carvalho

2009

Rheumatol Int

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The main objective of this study was to compare clinical and laboratory data obtained from patients with primary antiphospholipid syndrome (PAPS) with and without Sneddon's syndrome (SS). A transverse study with 54 (85.2% female) PAPS patients (Sapporo criteria) was performed. Demographic, drug use, and antiphospholipid antibodies data were evaluated, as well as clinical and laboratory findings of SS. Patients were subdivided into one of two groups: PAPS with SS and PAPS without SS. Both groups were similar with respect to age (p = 0.05), gender (p = 0.34), race (p = 0.31), weight (p = 0.93), height (p = 0.27), and body mass index (p = 0.75); however, the SS group exhibited higher disease duration (96.0 ± 54.9 vs. 55.2 ± 52.0 months, p = 0.01). By definition, all PAPS with SS patients suffer from stroke, an arterial event; the frequency of stroke events (28.5 vs. 7.5%, p = 0.04), as well as of limb ischemia (100 vs. 30.0%, p < 0.0001) was higher in this group than in the PAPS without SS group. On the other hand, patients in the PAPS without SS group had more venous events, such as deep venous thrombosis, than those in the PAPS with SS group (80.0 vs. 50.0%, p = 0.03). In conclusion, an understanding of the relationship between APS and SS is important in order to identify a subgroup for which more rigorous accompaniment and therapy may be necessary.

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“…Due to our clinical experience the spectrum of differential diagnoses in patients admitted to us for second opinion in evaluation of PCNSV consists of noninflammatory vasculopathy due to arteriosclerosis [18] or drugs [33], Moyamoya disease and syndrome [24] and Sneddon´s syndrome [20][21][22]25] as well as atypical cases of multiple sclerosis [19]. One other important differential diagnosis of PCNSV is bacterial endocarditis [4], because both conditions may present with similar clinical and auxiliary findings but effort a different treatment regimen [5].…”

Section: Discussionmentioning

confidence: 99%