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March 1982

Acneform Eruption in Apert's Syndrome: Acrocephalosyndactyly

Author Affiliations

From the Department of Dermatology, Loma Linda (Calif) University School of Medicine. Dr Steffen is now with the Palm Springs (Calif) Medical Center.

Arch Dermatol. 1982;118(3):206-208. doi:10.1001/archderm.1982.01650150068029

Apert's syndrome (acrocephalosyndactyly) is inherited in an autosomal dominant fashion and is characterized by synostosis of the feet, hands, carpi, tarsi, cervical vertebrae, and skull. Craniofacial deformity results from early and irregular obliteration of the cranial sutures. The cranium is broad and high, with the highest point of the vault near or anterior to the bregma. The occiput is generally inconspicuous, and the back of the head is flattened. The forehead is prominent and more vertical than is normal. Swelling of the bregma results in a deep, transverse groove above the supraorbital fissures. The maxilla is hypoplastic, the face flattened, the upper jaw retrognathic, and the lower jaw relatively prognathic. Protrusion of the eyeballs, increased distance between the eyes (ocular hypertelorism), and strabismus are frequently notable. The outer canthi lie on a lower plane than the inner canthi, giving an outward and downward slant to the palpebral fissures. The nose