Epidermodisplazia Verrusiformis Tanısı Konulan İki Kardeş: Olgu Sunumu Epidermodisplazia Verrusiformis

Yıl 2019, Cilt: 17 Sayı: 3, 435 - 441, 02.12.2019

Tekden Karapınar Hilal Kaya Erdoğan İşıl Bulur Zeynep Nurhan Saraçoğlu Emine Dündar

Öz

 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

Öz

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.

Anahtar Kelimeler

cutaneous malignancy, epidermodisplasia verrusiformis

Kaynakça

• 1-Castro-Perez GA, Sorin I, Bravo AI, Mazzuoccolo LD. Acquired epidermodysplasia verrusiformis in a patient with congenital HIV infection. Actas Dermosifiliogr 2013; 104:731-3.
• 2-Rogers HD, Macgregor JL, Nord KM et al. Acquired epidermodysplasia verruciformis. J Am Acad Dermatol. 2009;60:315-20
• 3-Kivanc-Altunay I, Erdogan HK, Kayaoglu S. Perianal warts and the development of squamous cell carcinoma in epidermodysplasia verruciformis. Indian J Dermatol Venereol Leprol. 2011;77:112.
• 4-Hirschman D, Tacastacas J, Rady PL, Tyring SK, Cooper K, Honda K. Acquired Epidermodysplasia Verruciformis Associated with Human Papilloma Virus Type 14 in a Small a Small BowelTransplanted Child-A Case Report. Pediatr Dermatol. 2016;33:e1-5
• 5-McDermott DF, Gammon B, Snijders Pjet al.Autosomal dominant epidermodysplasia verruciformis lacking a known EVER1 or EVER2 mutation. Pediatr Dermatol. 2009;26:306-10.
• 6- Yoshida R, Kato T, Kawase M, Honda M, Mitsuishi T.Two sisters reveal autosomal recessive inheritance of epidermodysplasia verruciformis: a case report. BMC Dermatol 2014;21;14:12.
• 7- Sirianant L, Ousingsawat J, Tian Y, Schreiber R, Kunzelmann K. TMC8 (EVER2) attenuates intracellular signaling by Zn2+ and Ca2+ and suppresses activation of Cl- currents. Cell Signal. 2014;26:2826-33
• 8- Boza JC, Peruzzo J, de Oliveira FB, Nazar FL, Bakos RM. Epidermodysplasia Verruciformis Verruciformis-like Skin Eruption in an HIV-positive Patient. Trop Med Health.2014;42:185.