An Egyptian boy with Haberland syndrome: Case report with observations on the histopathology

Abstract

Haberland syndrome is characterized by diverse malformations affecting the ecto-mesodermal tissues (the central nervous system, the eye, and the skin). The most prominent anomalies include a hairless fatty tissue nevus of the scalp (nevus psiloliparus) and ocular choristomas.1 In addition, several central nervous system (CNS) anomalies have been described, including cranial and spinal lipomas, partial or complete hemisphere atrophy, and intracranial cysts that can lead eventually to mental retardation and/or seizures.2 We present a case of Haberland syndrome that met the revised diagnostic criteria proposed by Moog in 20092 and can be considered a definite case of Haberland syndrome despite the absence of a fatty nevus. In addition, we demonstrate that the histopathologic findings of vascular hyperplasia are a prominent feature of all involved sites.