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September 1978

Hemifacial Atrophy: A Review of an Unusual Craniofacial Deformity With a Report of a Case

Arch Otolaryngol. 1978;104(9):538-541. doi:10.1001/archotol.1978.00790090056012

• Progressive hemifacial atrophy is an unusual craniofacial deformity that is amenable to surgical reconstruction. The disease may involve any layer of the superficial facial tissues as well as cartilage and bone. While the cause of the disease is unknown, several hypotheses exist to account for the progressive nature of the disorder. Histologically, the skin undergoes thinning of the Malpighian stratum and atrophy of the skin adenexa and dermal units. The subcutaneous tissue shows chronic inflammatory changes. This picture closely resembles that of scleroderma, and some authors believe the disorder to be an isolated form of scleroderma. Various hypotheses have been promulgated as to the cause of this disorder, its pathologic process, and varied clinical presentation.

(Arch Otolaryngol 104:538-541, 1978)