Goltz Syndrome, A Rare Entity: A Case Report

Abstract

Goltz syndrome or focal dermal hypoplasia is a rare multiple system disorder with cutaneous, ocular, dental and skeletal abnormalities. Its hallmark is thinning of the dermis resulting in subcutaneous fat herniation. It is a type of ectodermal dysplasia, a group of heritable disorder causing hair, teeth, nail and glands to develop and function abnormally. Here in, we are presenting a case of this syndrome in a 9-year-old girl presenting with non-pruritic ill-defined depigmented and hyperpigmented macular lesion on both sides of flanks and on hands, since birth which was suspected to have Goltz syndrome on clinical grounds and confirmed subsequently on histopathological examination of skin biopsy.

Keywords: Focal dermal hyperplasia, Goltz syndrome, Jessner-Cole syndrome

Cite this Article

Suman, Kishore Khatri, Rajni Joshee et al. Goltz Syndrome, a Rare Entity: A Case Report. Research & Reviews: Journal of Oncology and Hematology. 2017; 6(2): 11–14p.