Tekden KARAPINAR, Hilal Kaya ERDOĞAN, İşıl BULUR, Zeynep Nurhan SARAÇOĞLU, Emine DÜNDAR

Epidermodisplazia Verrusiformis Tanısı Konulan İki Kardeş: Olgu Sunumu Epidermodisplazia Verrusiformis

Epidermodisplazis verrusiformis (Lewandowsky Lutz sendromu) nadir görülen, otozomal resesif geçen, hücresel immunitedeki defekt ile karakterize ve Human Papillomavirüs enfeksiyonuna duyarlılığın arttığı bir genodermatozdur. Tipik klinik bulgular arasında pitriyazis versikolora benzer maküller, düz siğil benzeri papüller, psöriaziform kırmızı papüller ve seboreik keratoza benzeyen pigmente keratotik lezyonlar bulunur. Güneş gören bölgelerde kütanöz maligniteler gelişebilmektedir. Burada Epidermodisplazis verrusiformis tanısı konulan iki kardeş olgu sunulmaktadır. Kardeşlerden biri 13 yaşında kız, diğeri ise 15 yaşında erkekti. El, yüz ve boyunlarında beş yıldan beri olan cilt lezyonları mevcuttu. Epidermodispazia verrusiformis hastalarında özellikle güneş gören bölgelerde, erken yaşlarda kütanöz maliniteler gelişebilmektedir. Bu nedenle hastaların güneşten korunmasının ve dermatologlar tarafından yakın takibinin uygun olduğunu düşünmekteyiz.

Anahtar Kelimeler:

epidermodisplazis verrusiformis, Human Papillomavirüs

Two siblings diagnosed with epidermodysplasia verrusiformis: Case report Epidermodysplasia verrusiformis

Epidermodisplasia verrusiformis (Lewandowsky Lutz syndrome) is a rare, autosomal recessive genodermatosis characterized by a defect in cellular immunity and increased susceptibility to Human Papillomavirus infection. Typical clinical findings include pityriasis versicolor-like macules, flat wart-like papules, psoriasiform red papules, and pigmented keratotic lesions resembling seborrheic keratosis. Cutaneous malignancies can develop on sun-exposed regions. Herein, we present two siblings diagnosed with Epidermodisplasia verrusiformis. One of the siblings is a 13-year-old girl and the other is a 15-year-old. There were skin lesions on the hands, face and neck for five years. In patients diagnosed with Epidermodispazia verruculiformis, particularly in the sun-exposed areas, cutaneous malinites can develop in younger ages. For this reason, we think that it is appropriate for the patients to be protected from the sun and closely followed by the dermatologists.

Keywords:

cutaneous malignancy, epidermodisplasia verrusiformis,

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