Antiphospholipid-antibody-negative Sneddon's Syndrome: a case report and review of the literature/Otoantikorlari negatif Sneddon Sendromu: olgu sunumu ve literaturun gozden gecirilmesi

ABSTRACT

Sneddon's Syndrome is a systemic disease characterized by livedo reticularis and cerebrovascular lesions. Its clinical presentation varies widely and its etiology is still unknown. Other symptoms that can accompany are cognitive decline, epilepsy, headache, miscarriages and hypertension.

A 48-year-old female with a history of hypertension, 6 miscarriages and one stillbirth presented to our clinic with acute left-sided weakness and vision loss. Physical examination showed blue-purple skin marks with irregular borders on her upper and lower extremities. The patient's neurological examination revealed left homonymous hemianopia, mild hemiparesis of distal upper extremities and globally hyperactive deep tendon reflexes. Brain imaging confirmed a subacute right occipital infarct and showed chronic infarcts in the left frontal and temporal lobes, bilateral cerebellar infarctions and ischemic changes in the periventricular regions. Anticardiolipin antibodies and lupus anticoagulant were negative. Factor V Leiden mutation was not found, homocysteine, Protein S and protein C activity and fibrinogen levels were within the normal ranges. Titre of antinuclear antibodies (ANA) was over 1/100. Neuropsychological testing revealed mild memory difficulties. The skin biopsy was compatible with Sneddon's syndrome. The patient was diagnosed with Sneddon's syndrome based on historical, clinical and laboratory findings and antiaggregant therapy was initiated.

In this case report, we aimed to discuss clinical manifestations, pathological findings in the skin lesions and neuroradiological findings in a patient with antiphospholipid-antibody-negative Sneddon's syndrome and wanted to share our treatment approach. (Archives of Neuropsychiatry 2013; 50:180-182)

Key words: Sneddon's Syndrome, generalized livedo reticularis, cerebrovascular lesion, autoantibodies

Conflict of Interest: The authors reported no conflict of interest related to this article.

OZET

Sneddon Sendromu ciltte livedo retikularis ve tekrarlayan serebrovaskuler ataklarla karakterize, otoimmun oldugu dusunulen ve nadir gorulen bir hastaliktir. Tekrarlayan dusukler, nobet, kognitif yetmezlik, hipertansiyon hastalikta gorulen diger semptomlardir.

48 yasinda kadin hasta gorme bozuklugu, sol taraf gucsuzlugu sikayeti ile basvurdu. Ozgecmisinde hipertansiyon ve alti dusuk, bir olu dogum oykusu mevcuttu. Fizik muayenesinde her iki ust ve alt ekstremitede mavi-mor renkli duzensiz sinirli cilt lezyonlari mevcuttu. Norolojik muayenesinde sol homonim hemianopsi, her iki ust ekstremite distallerinde frust parezi ve derin tendon reflekslerinde canlilik saptandi. Beyin goruntulemesinde sag oksipital subakut enfarkt, sol temporal, her iki serebellar ve sol frontal kronik enfarkt alanlari ve periventrikuler iskemik degisiklikler mevcuttu. Antikardiolipin antikorlari ve lupus antikoagulani negatif, fibrinojen, homosistein, protein-C, protein aktivitesi normal sinirlardaydi, faktor 5 Leiden mutasyonu saptanmadi, ANA 1/lOOtitrede pozitif idi. Noropsikometrik testte orta derecede bellek bozuklugu vardi. Lezyonlu bolgeden yapilan cilt biyopsisinde Sneddon Sendromuyla uyumlu biyopsi bulgulari saptandi. Ozgecmis, klinik ve laboratuvar sonuclari ile Sneddon Sendromu tanisi dusunulen hastaya antiagregan tedavisi baslandi.

Bu olgu sunumunda Sneddon Sendromunun tipik bulgularini gosteren ve otoantikorlari negatif saptanan bir hastanin klinik gostergeleri, cilt biyopsisi ve nororadyolojik bulgularinin tartisilmasi ve tedavi yaklasimimizin paylasilmasi amaclanmistir. (Noropsikiyatri Arsivi 2013; 50:180-182)

Anahtar kelimeler: Sneddon Sendromu, jeneralize livedo retikularis, serebrovaskuler lezyon, otoantikor

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Giris

Sneddon Sendromu (SS),...