Cognitive and psychiatric changes as first clinical presentation in Sneddon Syndrome / Alterações cognitivas e psiquiátricas como primeira apresentação clínica na Síndrome de Sneddon

ABSTRACT Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

RESUMO A síndrome de Sneddon (SS) é uma vasculopatia trombótica não inflamatória progressiva rara que afeta os vasos sanguíneos de pequeno e médio tamanho com origem desconhecida. Está fortemente associada à presença de anticorpos antifosfolipídios (AA). A presença de livedo reticularis e doença cerebrovascular são a marca registrada. É muito mais comum em mulheres jovens. Relatamos um caso de SS em um homem de 43 anos de idade com dois anos de história de comprometimento cognitivo progressivo compatível com síndrome demencial e mudanças graves de personalidade, além de livedo reticular e padrão angiográfico cerebral de vasculite. AA eram limítrofes. O reconhecimento das manchas da pele que precedem eventos isquêmicos cerebrovasculares deve reforçar a hipótese de SS. Os AA são elevados em mais da metade dos casos, mas seu papel na patogênese ou associação de anticorpos positivos e SS permanece obscuro. A síndrome demencial em pacientes jovens deve ser amplamente investigada para se descartarem situações reversíveis. Achados típicos da pele, ressonância magnética e angiografia podem ajudar no diagnóstico.

Síndrome de Sneddon asociado a síndrome antifosfolipídico: descripciones clínicas y revisión de la literatura / Sneddon syndrome associated with antiphospholipid syndrome: clinical descriptions and a literature review

Resumen El síndrome de Sneddon es una rara vasculopatía no inflamatoria, obliterante, caracterizada por la asociación de eventos cardiovasculares (hipertensión arterial, claudicación intermitente y enfermedad coronaria), neurológicas (accidentes cerebrovasculares isquémicos, cefalea, vértigo y convulsiones) y livedo reticularis de tipo racemosa. Presentamos a una mujer que ingresa con un cuadro neurológico isquémico, hipertensión arterial, problemas vasculares y lesiones en piel. La biopsia de piel se catalogó como dermatitis perivascular superficial linfocitaria, sugestivo de lesión oclusiva.

Abstract Sneddon syndrome is a rare non-inflammatory obliterative vasculopathy, characterised by the association of cardiovascular (arterial hypertension, intermittent claudication, and coronary artery disease) and neurological events (ischaemic stroke, headache, dizziness and convulsions), and livedo reticularis/livedo racemosa. The case is presented of a woman admitted with an ischaemic neurological disease, hypertension, vascular problems, and skin lesions. The skin biopsy was classified as surface perivascular lymphocytic dermatitis, suggestive of occlusive lesion.

Síndrome de Sneddon: reporte de un caso / Sneddon syndrome: case report

El síndrome de Sneddon es una vasculitis sistémica, que se caracteriza por accidente cerebrovascular isquémico y livedo reticularis. Es una enfermedad rara, que se da principalmente en mujeres jóvenes. Presentamos el caso de una mujer de 20 años con el diagnóstico de Síndrome de Sneddon, tratada en forma oportuna y con una evolución favorable en el hospital Doctor Hernán Henríquez Aravena de Temuco (HHHA).

Sneddon's syndrome is a systemic vasculitis, characterized by ischemic stroke and livedo reticularis. It is a rare disease that occurs mainly in young women. We report the case of a 20 year old woman diagnosed with Sneddon syndrome, treated in time and with a favorable outcome at Dr. Hernán Henríquez Aravena Hospital (HHHA) of Temuco

Acute Central Retinal Artery Occlusion Associated with Livedoid Vasculopathy: A Variant of Sneddon's Syndrome

Livedoid vasculopathy (LV) is characterized by a long history of ulceration of the feet and legs and histopathology indicating a thrombotic process. We report a case of acute central retinal artery occlusion in a 32-year-old woman who had LV. She showed no discernible laboratory abnormalities such as antiphospholipid antibodies and no history of cerebrovascular accidents. Attempted intra-arterial thrombolysis showed no effect in restoring retinal arterial perfusion or vision. The central retinal artery occlusion accompanied by LV in this case could be regarded as a variant form of Sneddon's syndrome, which is characterized by livedo reticularis and cerebrovascular accidents.

Sneddon's syndrome: case report and review of its relationship with antiphospholipid syndrome / Síndrome de Sneddon: relato de caso e revisão sobre a relação com a síndrome do anticorpo antifosfolipídio

The Sneddon's syndrome is a rare disorder characterized by the occurrence of cerebrovascular disease associated with livedo reticularis. The antiphospholipid syndrome is the most frequent type of acquired thrombophilia, defined by the occurrence of thrombosis or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. These antibodies may play a pathogenetic role in some cases of Sneddon's syndrome, and many authors consider these two syndromes as the same entity. Although clinical features of antiphospholipid syndrome and Sneddon's syndrome may overlap, there is a distinction between clinical and laboratory evidence suggesting that these two entities are different diseases. A recent finding of coagulopathies, including elevated levels of coagulation factor VII, decreased levels of protein S, and activated protein C in Sneddon's syndrome patients suggested a possible biological link between the vasculopathy and a primary coagulopathy. Moreover, the clinical course seems to be progressive in Sneddon's syndrome patients and includes increase of disability and cognitive deterioration, more arterial involvement, and the antiphospholipid syndrome shows a more benign course. Both syndromes share clinical and laboratory features, and whether Sneddon's syndrome represents a spectrum of antiphospholipid syndrome remains unclear. Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. It is important to recognize the Sneddon's syndrome, particularly because stroke episodes may be prevented through appropriate treatment.

A síndrome de Sneddon é um distúrbio raro caracterizado pela ocorrência de doença cerebrovascular associada a livedo reticular. A síndrome do anticorpo antifosfolipídio é o tipo mais frequente de trombofilia, definida pela ocorrência de trombose ou morbidade gestacional na presença de anticorpos antifosfolípides persistentemente positivos. Aproximadamente 80% dos pacientes com síndrome de Sneddon apresentam um marcador de anticorpo antifosfolipídio. Esses anticorpos podem exercer um papel fisiopatológico em alguns casos de síndrome de Sneddon, e muitos autores consideram essa síndrome e a síndrome do anticorpo antifosfolipídio a mesma entidade. Apesar de os quadros clínicos das suas síndromes poderem se sobrepor, há evidência clínica e laboratorial distintiva, sugerindo que as duas entidades são doenças diferentes. Um achado recente de coagulopatia, incluindo níveis elevados do fator VII de coagulação, diminuição dos níveis da proteína S, e proteína C ativada em pacientes com síndrome de Sneddon, sugeriu uma possível ligação biológica entre a vasculopatia e coagulopatia primária. Além disso, o curso clínico pareceu ser progressivo em pacientes com síndrome de Sneddon, visto que há aumento de incapacidade e deterioração cognitiva, além de maior envolvimento arterial, enquanto a síndrome do anticorpo antifosfolipídio apresenta um curso mais benigno. Ambas as síndromes compartilham características clínicas e laboratoriais; até qual ponto a síndrome de Sneddon representa um espectro da síndrome do anticorpo antifosfolipídio permanece desconhecido. Os pacientes com a primeira síndrome apresentam pior prognóstico e podem representar um subgrupo de pacientes que requer um seguimento mais rigoroso. É importante reconhecer a síndrome de Sneddon já que os episódios de acidente vascular cerebral podem ser prevenidos com a terapia apropriada.

Sneddon syndrome associated with Protein S deficiency.

Sneddon syndrome (SS) is rare, arterio-occlusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown. Seizure, cognitive impairment, hypertension, and history of repetitive miscarriages are the other symptoms seen in this disease. Livedo racemosa involves persisting irreversible skin lesions red or blue in color with irregular margins. Usually, SS occurs in women of childbearing age. Protein S deficiency is an inherited or acquired disorder associated with an increased risk of thrombosis. We present a 33-year-old woman with SS with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Protein S deficiency and with Sneddon syndrome rarely encountered in the literature.

A Case of Sneddon's Syndrome / 대한피부과학회지

Sneddon's syndrome is an infrequent neurocutaneous disorder of unknown origin. It is characterized by the combination of livedo reticularis and cerebrovascular accident. We present a 57-year-old male patient with livedo reticularis and cerebrovascular accident. Magnetic resonance imaging of the head showed a sign of acute focal infarctions in the right cerebellar hemisphere and right vermis. He had netlike patterned, mottled bluish discoloration on both legs. Histopathologic finding revealed elongation and fusion of rete ridges and mild thickening of dermal capillaries.

Sneddon's Syndrome

Sneddon's syndrome consists of widespread livedo reticularis and ischemic cerebral manifestations. We report a case of a 70-year-old Korean woman with extensive livedo reticularis, hypertension, positive laboratory results for antinuclear antibodies and the lupus anticoagulant test, and idiopathic Parkinson's disease.

A Case of Sneddon's Syndrome / 대한피부과학회지

Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions with unknown etiopathogenesis. We report a 16-year-old male patient who had been suffered from livedo reticularis for 10 years and multiple cerebral infarction for 2 years. Although the histologic finding of the skin did not show vasculitis and vascular occlusions, the patient revealed livedo reticularis and central nervous system symptoms. These findings were compatible with Sneddon's syndrome.