Article
Poikiloderma with Neutropenia in Morocco: a Report of Four Cases.
Journal of clinical immunology - 1 May 2017
Aglaguel Ayoub, Abdelghaffar Houria, Ailal Fatima, Habti Norddine, Hesse Sebastian, Kohistani Naschla, Klein Christoph, Bousfiha Ahmed Aziz
Abstract excerpt
PURPOSE: Poikiloderma with Neutropenia (PN) is inherited genodermatosis which results from a biallelic mutation in the USB1 gene (U Six Biogenesis 1). PN, first described in Navajo Native Americans, is characterized by early onset poikiloderma, pachyonychia, palmo-plantar hyperkeratosis, and permanent neutropenia. This condition results in frequent respiratory tract infections during infancy and childhood. From...
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