Dowling-Degos disease:  clinical and histopathological findings of 6 cases

Mansur, Ay�e T�lin; G�nd�z, Sevil; Uygur, T�lin; Akg�n, Nil�fer; Aker, F�gen

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Dowling-Degos disease: clinical and histopathological findings of 6 cases [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2004; 38(2): 126-133

Dowling-Degos disease: clinical and histopathological findings of 6 cases

Ay�e T�lin Mansur¹, Sevil G�nd�z¹, T�lin Uygur¹, Nil�fer Akg�n², F�gen Aker³
¹S.b. Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Deri Hastal�klar� Klini�i
²S.b. Beykoz Devlet Hastanesi Dermatoloji Poliklini�i
³S.b. Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Patoloji B�l�m�

Background and design: Dowling-Degos disease (DDD) is a rare genodermatosis with an autosomal dominant inheritance pattern, characterized by reticular hyperpigmentation, hyperkeratotic follicular lesions and perioral pitted scars. In recent years, it is postulated that a group of different genodermatoses including reticulate acropigmentation of Kitamura (RAK), are different expressions of a single disease and should be regarded in a spectrum of a complex entity. MATERIALS-METHODS: Here, 6 cases of DDD, which 4 of them also showing some features characterizing RAK, are presented. All the patients were women, and the average age of disease onset was found to be 21.2. RESULTS: Three cases had family history of similar pigmentation. In 1 case, lesions were confined to vulva, 2 of the other cases showed palmoplantar keratoderma which has not been described with DDD before. Three cases demonstrated soft fibromas, 1 case had active lesions of furoncules and 2 additional cases described furonculosis in their family. The histopathological examinations revealed typical findings of DDD and RAK in all patients.

Keywords: Dowling-Degos disease, reticular acropigmentation of Kitamura, reticular pigmentation, pigmentation disorder

Dowling-Degos Hastal��: 6 Olgudaki Klinik ve Histopatolojik Bulgular

Ay�e T�lin Mansur¹, Sevil G�nd�z¹, T�lin Uygur¹, Nil�fer Akg�n², F�gen Aker³
¹S.b. Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Deri Hastal�klar� Klini�i
²S.b. Beykoz Devlet Hastanesi Deri ve Z�hrevi Hastal�klar Poliklini�i
³S.b. Haydarpa�a Numune E�itim Ve Ara�t�rma Hastanesi Patoloji B�l�m�

Dowling-Degos hastal�� (DDH), esas olarak fleksural b�lgeleri ilgilendiren retik�ler hiperpigmentasyon, hiperkeratotik follik�ler lezyonlar ve a��z �evresinde ��k�k noktasal skarlar ile karakterize, otozomal dominant ge�i�li, nadir g�r�len bir genodermatozdur. Son y�llarda ba�ta Kitamura�n�n retik�ler akropigmentasyonu (KRA) olmak �zere ba�ka baz� tablolar�n da bu hastal��n de�i�ik g�r�n�mleri olabilece�i ve bunlar�n bir spektrum i�inde de�erlendirilmeleri gerekti�i belirtilmektedir. Bu yaz�da 4�� KRA�ya ait baz� bulgulara da sahip olan 6 DDH olgusunun klinik ve histopatolojik �zellikleri belirtilmi�tir. Hepsi kad�n olan olgularda hastal��n ba�lang�� ya�� ortalama 21.2 olarak hesaplanm��, 3 olguda aile �yk�s� pozitif bulunmu�tur. Olgular�n 1�inde sadece vulva tutulumu g�r�lm��, 2 olguda ise daha �nce DDH i�in bildirilmemi� olan palmoplantar keratoderma saptanm��t�r. Olgular�n 3��nde yumu�ak fibromlar, 1 olgunun kendisinde, 2 olgunun ise ailesinde fronk�loz varl�� dikkati �ekmi�tir. T�m olgularda DDH veya KRA i�in tan�mlanan tipik histopatolojik �zelliklere rastlanm��t�r.

Anahtar Kelimeler: Dowling-Degos hastal��, Kitamura�n�n retik�ler akropigmentasyonu, retik�ler pigmentasyon, pigmentasyon bozuklu�u

Ay�e T�lin Mansur, Sevil G�nd�z, T�lin Uygur, Nil�fer Akg�n, F�gen Aker. Dowling-Degos disease: clinical and histopathological findings of 6 cases. Turkderm-Turk Arch Dermatol Venereol. 2004; 38(2): 126-133

Corresponding Author: Ay�e T�lin Mansur, T�rkiye

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