Pamir GÜLEZ, Murat HIZARCIOGLU, Ertan KAYSERILI, Sule AFSAR, Ragıp ORTAÇ, Hursit APA

ÇOCUKLUK ÇAĞININ KRONİK BÜLLÖZ HASTALIĞI TANILI BİR OLGUDA İNTRAVENÖZ İMMUNGLOBULİN TEDAVİSİ

Çocukluk çağının kronik büllöz hastalığı nadir görülen, etiyolojisi tam olarak bilinmeyen subepidermal büllühastalıklardandır. Bu hastalıkta direkt immunofloresan yöntemi ile epidermal bazal membranda IgA'nın lineerdepolanması patognomoniktir. Tedavide sıkılıkla dapson kullanılır. İntravenöz immunglobulin tedavide yeni biryaklaşımdır. Oniki aylık erkek hasta özellikle ağız çevresinde olmak üzere yüzünde, kalçasında, kol vebacaklarında çok sayıda bül varlığı yakınması ile başvuru üzerine hastaneye yatırıldı. Cilt biyopsi örneğinindirekt immunofloresan yöntemiyle incelenmesinde dermoepidermal hat boyunca IgA depolandığı görüldü. Bubulgularla olguya çocukluk çağı kronik büllöz hastalığı tanısı konuldu. Olguda tedavi amacıyla intravenözimmunglobulin kullanıldı ve üç gün içinde tüm lezyonların tamamen iyileştiği belirlendi. Hasta bir yıldırherhangibir tekrarlama görülmeksizin takip edilmektedir

Anahtar Kelimeler:

Çocukluk çağının kronik büllöz hastalığı, immunglobulinA, intravenöz immunglobulin

Intravenous Immunglobulin Therapy In A CaseWith Chronic Bullous Disease of Childhood

Chronic bullous disease of childhood, one of the subepidermal bullous diseases is uncommon, and its etiology has not fully understood. A linear deposition of IgA at the epidermal basement membrane on direct immunoflorescence is pathognomonic for the disease. Dapson is commonly used in the therapy. Intravenous immunglobulin is a recent choice in the treatment. A twelve-months old boy was admitted to the hospital with the complaints of mutipl blisters on his face, especially perioral region, buttocks and extremities. Examination of the skin biopsy specimen with direct immunoflorescence method showed linear deposits of IgA along the dermoepidermal junction. In the light of the findings, condition was diagnosed as chronic bullous disease of childhood. Following the intravenous immunglobulin use for treatment, the skin lesions revealed rapid healing within three days.

Keywords:

Chronic bullous disease of childhood, immunglobulinA, intravenous immunglobulin,

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