SNEDDON SYNDROME: A CASE REPORT

ANA LETÍCIA XAVIER AMARAL, LUÍSA SOUZA ECHEVERRIA, BEATRIZ GON PEREZ NARDOQUE, BIANCA RIBEIRO MORAIS, ROBERTA EDUARDA COCCENELLO

Resumo


Introduction Snelddon Syndrome (SS) is a rare disturb characterized by ischemic cerebrovascular accident (CVA) associated with livedo reticularis. About 80% of the patients with SS also present the Antiphospholipid Syndrome (APS). Even though some authors classify the two syndromes as equals, there are clinical and laboratorial differences between them. It is important to recognize the Snelddon Syndrome at its beginning in order to avoid cognitive deterioration and incapacity Case report: A 55 years old woman, admitted at the Hospital Universitário-UFGD in December of 2016 presenting conscience level diminishing related to aspirative pneumonia. Previous history of CVA with the last episode in 2013 with evolution to a demential state. At physical exam, verbal response with incomprehensible sounds and asymmetric spastic tetra paresis with predominance of the left side. Reticular violet erythematous lesions in MMII, trunk, dorsum and abdomen; Raynaud phenomenon in extremities, necrosis in distal phalanges of second, third and fourth left podotactiles and in the second right podotactile digital pulp.The patient tested negative for syphilis, hepatitis B, C and HIV. Transthoracic echocardiography: moderated to important mitral insufficiency and thickened aortic valve.Cranial tomography presented diffuse cerebral atrophy with no lobar predominance, diffuse hypo density of the periventricular white matter and corona radiate, consistent with microangiopathy. Furthermore, the right lobe parieto occipital and left lobe parietal hypodensity were consistent with ischemic territorial CVA. Patient was hypertensive and took drugs regularly to control blood pressure. In order to investigate non inflammatory thrombotic vasculopathy, the levels of antipospholipid antibodies were measured. The patient tested reagent for lupus anticoagulant (LA), IgM anti-beta 2 glycoprotein non reagent, IgG reagent, IgM anticardiolipin antibody non reagent and IgG with moderate reativity. Patient was diagnosed with SS with antiphospholipid antibodies. Discussion: The SS characterizes by the association of livedo reticularis (LR) and thrombotic vasculopathy. Usually, the LR anticipates a cerebrovascular disease and presents as violet erythematous lesions most common in members and not related to cold. Other clinical presentations are hypertension, migraine, dementia, cardiac valvulopathies and kidney diseases. Furthermore, acrocyanosis, Raynaud phenomenon and cutaneous necrosis might be dermatological findings of SS. SS is a long lasting disease and patients can present cognitive disturbs and deterioration throughout time. High levels of the VII coagulation factor, diminishing of the S protein and protein C resistance in SS can differ it from APS. In this case report, the SS diagnosis was made after previous history of CVA, tomographic analysis, LR confirmation and laboratorial exams which confirmed the presence of APS.

Referências


References: Vladimir Arruda Zaccariotti; Luiz Fernando Martins; Valter da Costa; Nilzio A. da Silva; Alberto A. Las Casas; Sebastião Eurico de Melo-Souza. Sneddon's syndrome: report of three cases. Arq. Neuro-Psiquiatria. São Paulo, v.53, n.1,p. 82-87, Mar. 1995.

DUTRA, Lívia A.; NETO, Pedro B.; PEDROSO, José L.; BARSOTTINI, Orlando G. P. Síndrpme de Sneddon: relato de caso e revisão sobre a relação com a syndrome do anticorpo antifosfolipídico. Einstein, 2012; 10(2): 230-2.

W. Shengjun, X. Ziqi, L. Hui. Sneddon’s syndrome: a comprehensive review of the literature. Orphanet J Rare Dis 2014; 9:215.


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