Sneddon syndrome in Child
The key cutaneous feature is a persistent livedo reticularis, often in a livedo racemosa pattern (a form of livedo reticularis consisting of a large branching pattern, usually on the trunk, limbs, and proximal extremities). Individuals with Sneddon syndrome, including children, may have antiphospholipid antibodies. The prevalence has been reported to be from 0% to 85%. Regardless of whether patients have antiphospholipid antibodies, individuals with Sneddon syndrome have occlusive arteriopathy and endothelial damage. Up to 35% will have antiendothelial cell antibodies.
- Livedo racemosa – Often precedes the onset of focal neurologic symptoms.
- Headache and vertigo – Most frequent symptoms; may precede livedo racemosa by several years but are nonspecific.
- Ischemic strokes – Most symptoms are from middle cerebral artery infarcts and thalamic infarcts: hemiparesis, sensory alterations, and aphasia. Less commonly, there may be dysarthria, visual field deficits, or sudden spontaneous falls.
- Transient ischemic attacks
- Vascular dementia
- Secondary symptoms and other organ manifestations in children include growth and intellectual disability.
- Moyamoya disease – In childhood, presents with ischemic attacks or strokes with occluded intra- and extracerebral arteries. Skin manifestations are not common symptoms of the disease.
M30.8 – Other conditions related to polyarteritis nodosa
238776001 – Sneddon syndrome
- Cutis marmorata telangiectasia congenita – Present at birth, often limited to a limb that is hypotrophic, and may have ulceration.
- Livedoid vasculopathy – Painful punched-out ulcers around ankles; tends to affect middle-age and elderly individuals.
- Polyarteritis nodosa – Cutaneous variant (more common in children) is more likely to have livedo racemosa and nodules. Histology: leukocytoclastic vasculitis.
- Cholesterol emboli or atrial myxoma emboli – Characteristic histology shows cholesterol clefts within thrombi with cholesterol emboli.
- Necrotizing livedo reticularis – May involve cutaneous nodules and ulcerations. This has many disease associations, including leukocytoclastic vasculitis, calciphylaxis, cryoglobulinemia, granulomatosis with polyangiitis, atrial myxomas, Graves disease, connective tissue diseases, acute lymphocytic leukemia, and renal cancer.
- Amantadine-induced livedo reticularis – Usually occurs on the legs.
- Primary antiphospholipid antibody syndrome with livedo reticularis is associated with epilepsy.
- Systemic lupus erythematosus (SLE) with livedo reticularis is associated with more severe SLE.
- Essential thrombocythemia
- Thromboangiitis obliterans – Rest pain, chronic ulcers on distal extremities, gangrene of fingers and/or toes.
- Moyamoya syndrome – Shows stenosis or occlusion of extracranial and intracranial blood vessels.
- Malignant atrophic papulosis (Degos disease) – May exhibit some clinical features that overlap with those of Sneddon syndrome (eg, cerebrovascular accident in a young person without other known risk factors).