Sneddon’s syndrome-like manifestation as a presentation of abdominal aortic aneurysm thrombosis
Year: 2024
Type: article
Abstract: Dear Editor, Sneddon’s syndrome (SS) is a rare thrombotic vasculopathy characterized by a combination of livedo racemosa (LR) and cerebrovascular diseases, such as ischemic stroke or transient ischemic attack, particularly in the territory of the middle cerebral artery.[1,2] Literature reviews on SS with spinal strokes have also been documented.[3] Although it was once considered a clinical diagnosis, SS is now regarded as a common clinical manifestation of different disease entities. It can be idiopathic or associated with other etiological factors such as antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE).[4] Herein, we report an unusual case with SS-like presentation secondary to thrombosis of abdominal aortic aneurysm. A 63-year-old man with no significant past medical history presented with a sudden onset of low back pain radiating down both legs, followed immediately by bilateral lower limb paralysis with numbness. A sudden development of an extensive skin rash was also noted at the same time. Dermatological examination revealed multiple irregularly broken, netlike mottled violaceous patches involving the lower limbs, buttocks, and mid/lower trunk circumferentially, consistent with LR [Figure 1]. Neurological examination revealed decreased muscle power and temperature sensation in bilateral lower limbs, with a sensory level below T10 on pinprick sensation. Increased upper motor neuron signs were also noted. The neurological symptoms and signs suggested ischemia of the anterior spinal cord.Figure 1: Clinical manifestation. A 63-year-old man showed sudden development of multiple irregularly broken, net-like mottled violaceous patches on the mid/lower trunk (a), and lower limbs (b).The computed tomography scan of the abdomen disclosed a 6.5-cm diameter infrarenal abdominal aortic aneurysm with sac thrombosis [Figure 2a]. Laboratory investigations, including antiphospholipid (aPL) antibodies, antinuclear antibodies, and lupus anticoagulants, produced negative results. There was no evidence of vasculitis, cryoglobulinemia, or other autoimmune diseases. A skin biopsy taken from the white center of a livedo lesion on the right thigh showed fibrin thrombi in deep dermal arterioles [Figure 2b]. The final diagnosis was LR and spinal stroke secondary to thrombosis of abdominal aortic aneurysm, mimicking the manifestation of SS. The patient’s skin lesions and neurological symptoms improved gradually after endovascular repair of the abdominal aortic aneurysm, followed by antiplatelet therapy with aspirin 100 mg per day.Figure 2: Computed tomography and pathology. (a) Computed tomography scan revealed a 6.5-cm diameter infrarenal abdominal aortic aneurysm. (b) Histopathologic examination showed fibrin thrombi in dermal arterioles (H and E, ×400).SS is a rare disorder typically characterized by generalized LR and focal neurological deficits.[5,6] The incidence of SS is approximately four per million per year in the general population. Approximately 80% of the SS patients are young women. The etiology of SS is often unknown, and around half of the cases are idiopathic. SS is sometimes associated with autoimmune diseases such as SLE and APS, and aPL antibody markers are present in approximately 9%–50% of patients with SS. The frequent occurrence of aPL may suggest that SS is part of the clinical spectrum of the primary APS.[4,5,7-9] Laboratory investigations for autoimmune diseases or aPL markers in our patient were negative or returned results within normal limits. LR, the dermatological feature of SS, may precede the onset of neurologic ischemic event by years. It usually arises on the lower back and buttocks initially, and then, gradually advances to affect dorsal aspects of the arms and thighs. The anatomical distribution of LR in our case is unique, exhibiting a distinct demarcation below the abdomen. Another notable point is the simultaneous onset of LR and spinal stroke in our case. The thrombosis of aortic aneurysm could be the origin of thromboembolic occlusion affecting both the deep dermal arterioles and the artery supplying the anterior spinal cord, thereby resulting in the distinctive presentation observed in our case, different from the slowly progressive nature of classic SS. In conclusion, we reported a case exhibiting SS-like presentation with unusual dermatological and neurological manifestations resulting from thrombosis of abdominal aortic aneurysm. Prompt recognition of such symptoms is crucial to unveil hidden conditions and facilitate early intervention. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent form. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Data availability statement Data sharing not applicable to this article as no datasets were generated or analyzed during the current study. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Dermatologica Sinica
Authors: Shao-Heng Tai, Fang-Ying Wang, Shih-Jyun Yang
Language: English
Topic: Aortic Thrombus and Embolism
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $70
OpenAlex ID: W4401974057