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Updated: Aug 7, 2026

Isolation and Culture of Adult Epithelial Stem Cells from Human Skin
Published on: March 31, 2011
Hereditary woolly hair and keratosis pilaris
Andy J Chien1, Mark C Valentine, Virginia P Sybert
1Division of Dermatology, University of Washington, Seattle, Washington, USA. andchien@u.washington.edu
Abstract:
We describe a family with woolly hair and ulerythema ophryogenes spanning four generations. Both woolly hair and ulerythema ophryogenes have been associated with Noonan syndrome and cardiofaciocutaneous syndrome (CFC), two disorders with considerable phenotypic overlap. This family did not exhibit any of the other findings characteristic of either Noonan syndrome or CFC, similar to a previously described pedigree with hereditary woolly hair. Woolly hair elicits a broad differential diagnosis, including woolly hair nevus and several genodermatoses. Our report reviews the evaluation of woolly hair and discusses the conditions associated with this physical finding.
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