BACKGROUND: Sneddon syndrome is an occlusive vasculopathy that presents clinically with generalized livedo racemosa on the skin and transient ischemic attacks, strokes, and cognitive or motor deficits in the central nervous system. Antiplatelet or anticoagulant therapy is recommended. Due to the limited therapeutic efficacy and the resulting serious complications, we propose combination therapy with additional infusion cycles of alprostadil and captopril and report initial long-term results.
METHODS: We performed a systematic retrospective analysis of all patients with primary Sneddon syndrome who received combination therapy in our clinic between 1995 and 2020. Therapeutic outcomes were evaluated using descriptive statistics compared to historical controls receiving monotherapy. We also analyzed the event rate of complications when combination therapy was discontinued.
RESULTS: During the 99.7 patient-years of follow-up, there were no transient ischemic attacks and the stroke rate dropped to 0.02 per patient-year. In comparison, the rates of transient ischemic attacks and strokes in the historical controls ranged from 0.08 to 0.035 per patient-year. After discontinuation of alprostadil therapy, eight events occurred in three patients.
CONCLUSIONS: Combination therapy reduces the long-term incidence of ischemic events in patients with primary Sneddon syndrome.

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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.

暂无摘要。

OBJECTIVE: To report a case of occlusive retinal vasculopathy following varicella zoster infection in an immunocompetent adult.
METHODS: Observational case report.
METHODS: A patient with defective vision following chickenpox was evaluated with fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence.
RESULTS: Fundus showed multiple cotton wool spots and a well-demarcated zone of retinal ischemia in the posterior pole with normal optic disc without any evidence of anterior or posterior uveitis. Fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence findings revealed occlusive vasculopathy as the cause of defective vision.
CONCLUSIONS: We report a hitherto undescribed case of purely occlusive vasculopathy following varicella zoster infection without features of vasculitis or anterior and posterior uveitis in an immunocompetent individual.