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September 1967

Goltz's Syndrome: Focal Dermal Hypoplasia: A Combined Mesoectodermal Dysplasia

Author Affiliations

USA; USA, Denver
From the Dermatology and Allergy Service (Dr. Akers), and the Pediatric Service (Dr. Holden), Fitzsimons General Hospital, Denver. Dr. Holden is now at the US Public Health Service Hospital, New Orleans.

Am J Dis Child. 1967;114(3):292-300. doi:10.1001/archpedi.1967.02090240106009

FOCAL DERMAL HYPOPLASIA is a recently described syndrome1 which has a unique and characteristic clinical picture consisting of linear areas of dermal hypoplasia, abnormal skin pigmentation, and fawn-colored nodules of adipose tissue in association with other congenital anomalies. The 14 reported patients have all been girls. The purpose of this paper is to describe the 15th case and review this syndrome which is new to the pediatric literature.

Report of a Case  A 13-year-old white girl, the product of an eight-month gestation, weighed 1,361 gm (3 lb) at birth. The pregnancy was complicated by placenta previa but there were no infections, drug ingestion, or other complications. At birth a large omphalocele was present which was repaired at 2 days of age. Skin, eye, and musculoskeletal abnormalities were present at birth. Her skin at birth was similar to her present condition, but was more severely affected. There were small areas

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