A case report of two brothers with comedonal Darier�s disease

Mansur, A. T�lin; Ayd�ng�z, �kbal E.; Kocaayan, Nurhan; Er�in, Zuhal; Peker, �nder

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A case report of two brothers with comedonal Darier�s disease [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2006; 40(2): 72-74

A case report of two brothers with comedonal Darier�s disease

A. T�lin Mansur¹, �kbal E. Ayd�ng�z¹, Nurhan Kocaayan¹, Zuhal Er�in¹, �nder Peker²
¹Sb Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Dermatoloji Klini�i, �stanbul, Turkey
²Sb Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Patoloji B�l�m�, �stanbul, Turkey

Darier's disease is a genodermatosis affecting mainly the hair follicles and clinically presenting as hyperkeratotic papules arising mostly on the seborrheic areas like the trunk and the scalp. Comedonal Darier's disease is a rare type which is characterized by comedones and epidermoid cyst like lesions. In the literature 5 cases having these characteristics have been reported. Here is presented two brothers with 27 and 35 years of age, exhibiting the typical clinical features of Darier's disease in addition to many open and closed comedones and whitish-yellow cysts containing oily material, which are found on the face, especially the zygomatic prominences, axillae and the sternum. Histologic examination of these lesions showed cysts with lamellar type of keratin in the lumen and acantholysis, cleft formation and dyskeratotic cells in the epithelial wall of the cyst. The presence of comedonal Darier's disease in two brothers shows that hereditary and environmental factors may play a role in the development of this clinical variant of Darier's disease.

Keywords: Darier's disease, comedone, keratosis follicularis, cyst, epidermal cyst, comedonal Darier's disease

�ki Karde�te Komedonal Darier Hastal��

A. T�lin Mansur¹, �kbal E. Ayd�ng�z¹, Nurhan Kocaayan¹, Zuhal Er�in¹, �nder Peker²
¹Sb Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Deri ve Z�hrevi Hastal�klar Klini�i, �stanbul, T�rkiye
²Sb Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Patoloji B�l�m�, �stanbul, T�rkiye

Darier Hastal�� s�kl�kla k�l folik�llerini etkileyen, esas olarak g�vde ve sa�l� deri gibi seboreik b�lgeleri tutan, hiperkeratotik pap�llerle karakterize bir genodermatozdur. Komedonal Darier hastal�� ise komedonlar ve epidermoid kist benzeri lezyonlarla belirlenen nadir rastlanan bir formdur. Literat�rde bu �zellikleri ta��yan 5 olgu bulunmaktad�r. Bu makalede sunulan 27 ve 35 ya�lar�nda iki erkek karde�te, Darier hastal��n�n tipik klinik bulgular�na ek olarak y�zde �zellikle zigomatik b�lgelerde, koltuk altlar�nda ve sternum �zerinde �ok say�da a��k ve kapal� komedonlar, yer yer sar�-beyaz renkte, ya�l� i�erikli kistler bulunuyordu. Bu alanlardan yap�lan biyopsilerde izlenen kistik yap�lar�n duvar�n� d��eyen epitelde akantoliz, yar�k olu�umu ve diskeratotik h�creler, l�meninde ise lamellar tarzda keratin mevcuttu. Komedonal Darier hastal��n�n iki karde�te ortaya ��kmas�, bu klinik varyant�n geli�iminde kal�tsal veya �evresel fakt�rlerin rol oynayabilece�ini d��nd�rmektedir.

Anahtar Kelimeler: Darier hastal��, komedon, keratozis folik�laris, kist, epidermal kist, komedonal Darier hastal��

A. T�lin Mansur, �kbal E. Ayd�ng�z, Nurhan Kocaayan, Zuhal Er�in, �nder Peker. A case report of two brothers with comedonal Darier�s disease. Turkderm-Turk Arch Dermatol Venereol. 2006; 40(2): 72-74

Corresponding Author: A. T�lin Mansur, T�rkiye

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