Disseminated papular variant of Dowling‐Degos disease: Histopathological features in POGLUT1 mutation

Autor: Katharina, Papadopoulou, Syrus, Karsai, Almut, Böer-Auer
Rok vydání: 2022
Předmět:
Zdroj: JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 20:1423-1429
ISSN: 1610-0387
1610-0379
DOI: 10.1111/ddg.14897
Popis: Dowling-Degos disease is a rare benign genodermatosis. It is characterized by lentiginous hyperpigmentation and reddish-brown papules and plaques. The flexor sides and intertrigines are often affected, but the clinical appearance may vary. Mutations in different genes are responsible for the clinical manifestation. While mutations in the keratin 5 (KRT5) gene favor a reticular distribution pattern, mutations in the POGLUT1 gene lead to a disseminated, papular clinical picture. Acantholytic variants of Dowling-Degos disease have historically been referred to as Galli-Galli disease, but our case study shows that the histopathological changes can vary even within a single patient. To date, no standardized therapy concept exists. The main focus is on keratolytic measures, with varying response. New therapeutic approaches using laser technology appear to be a promising treatment option.
Databáze: OpenAIRE
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