A case of focal dermal hypoplasia.
- Author:
Myoung Ok KOH
;
Bong Kil JEON
;
Seok Don PARK
- Publication Type:Original Article
- Keywords:
Focal dermal hypoplasia;
Yellowish nodules
- MeSH:
Adipose Tissue;
Dermis;
Epidermis;
Extremities;
Female;
Fingers;
Focal Dermal Hypoplasia*;
Foot;
Humans;
Hypertrophy;
Leg;
Neck;
Skeleton;
Skin;
Telangiectasis;
Tooth;
Young Adult
- From:Korean Journal of Dermatology
1992;30(2):262-266
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Focal dermal hypoplasia (Goltz syndrome) is a rare genetic mesoectodermal disorder characterized by variable multisystem defects, including anomalies of the skin, skeleton, teeth and eyes. A 22-year-old female patient presented with confluent and disciete, yellowish nodules on the calves, white linear atrophic lesions, hypoand hyper!igmented patches, and telangiectasia scattered over the trunk, neck and extremities. In addition to the skin lesions, this patient showed hypertrophy of the left leg, anomalies of the left foot, fingers and nails, and maloccluded teeth. Histopathological findings of a yellow nodule revealed accumulations of adipose tissue very near to the epidermis that largely replaced the upper dermis.
