TUBERÖZ SKLEROZ VE İKTİYOZİS VULGARİS BİRLİKTELİĞİ: OLGU SUNUMU

Year 2009, Volume: 15 Issue: 1, 41 - 44, 01.03.2009

Selda Pelin Kartal Durmazlar Semih Tatlıcan Fatma Eskioğlu Hüseyin Üstün

Abstract

Tuberöz skleröz epilepsi, mental retardasyon, hamartomlar ve tipik deri bulguları ile karakterize nadir görülen otozomal dominant bir hastalıktır. Tuberöz skleroz tanısı olan 16 yaşındaki erkek hasta yüzündeki çok sayıda papüller nedeniyle görüldü. Fizik muayenede yüzde çok sayıda anjiofibromlar, gövdede hipopigmente maküller, lumbosakral bölgede shagreen patch ve fleksural bölgeler korunurken vücutta yaygın kepeklenme tespit edildi. Histopatolojik inceleme iktiyoz vulgaris ile uyumlu idi. Tuberöz sklerozda çeşitli deri bulguları olmasına rağmen iktiyozis vulgaris ile beraberliği daha önce bildirilmemiştir

Keywords

Tuberöz skleroz, Bourneville hastalığı, Epiloia, İktiyozis vulgaris

COEXISTENCE OF TUBEROUS SCLEROSIS AND ICHTHYOSIS VULGARIS: CASE REPORT

Abstract

Tuberous sclerosis is a rare autosomal dominant disorder. characterized by seizures, mental retardation, hamartomas and typical skin lesions. A 16-year old boy diagnosed with tuberous sclerosis was seen because of multiple papulles on his face. On physical examination multiple facial angiofibromas, hypopigmented macules on the body, shagreen patch on lumbosacral area and generalized scaling with flexural sparing were observed. Histopathology was consistent with ichthyosis vulgaris. Although tuberous sclerosis associates with various skin lesions, coexistence with ichthyosis vulgaris has not been reported previously

Keywords

Tuberous sclerosis, Bourneville disease, Epiloia, Ichthyosis vulgaris

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