Focal facial dermal dysplasia is a rare and genetically heterogeneous group of disorders, meaning that more than one genetic change can produce the same picture. The shared feature is a pair of scar-like lesions on both sides of the face that are present from birth, with or without other facial anomalies. The lesions are hairless and have a fingerprint-like puckering of the skin, especially at the temples, caused by alternating bands of dermal and epidermal atrophy. The group includes the conditions known as Brauer syndrome, also called bitemporal aplasia cutis congenita, and Setleis syndrome, also called facial ectodermal dysplasia. This entry records the condition as a named clinical entity and is not medical advice; diagnosis and care belong to a qualified clinician.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 1 Communicable or Non-communicable Causative Agent
Causative Agent (category) First Described Sources
1. Focal facial dermal dysplasia (Wikipedia)
Wikipedia lead
scar like facial lesions
Wikipedia lead [causative-agent-category]
genetically heterogeneous group of disorders
lead sentence 1
Focal facial dermal dysplasia is a rare genetically heterogeneous group of disorders that are characterized by congenital bilateral scar like facial lesions, with or without associated facial anomalies.
Lead: disease category
Focal facial dermal dysplasia is a rare genetically heterogeneous group of disorders that are characterized by congenital bilateral scar like facial lesions, with or without associated facial anomalies.
Article text
The syndrome was first described by Brauer in 1929 in a large five generation family (38 members).
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