Focal dermal hypoplasia. Current concepts and differential diagnosis.
Focal dermal hypoplasia syndrome (FDH) is a congenital disorder of mesodermal derivation with some ectodermal participation. The disorder, first described in 1962, is characterized by tenacious defects consisting of thinning of the skin; herniations of adipose tissue; abnormal skin pigmentation and other skeletal, dental, ocular, hair, and nail abnormalities. A rare instance of focal dermal hypoplasia syndrome is reported as characterized by papillary gingival hyperplasia. Differential diagnostic guidelines that separate focal dermal hypoplasia from a host of other skin and mucous membrane disorders are discussed along with therapeutic modalities and the possible relationship of human papillomavirus to focal dermal hypoplasia syndrome.
Greer
R O
RO
Division of Oral Pathology and Oncology, University of Colorado School of Dentistry, Denver.
Reissner
M W
MW
D000328:Adult; D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000473:pathology*; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000473:pathology*; D005885:Gingival Hyperplasia / Q000473:pathology*; D005887:Gingival Neoplasms / Q000473:pathology; D006801:Humans; D010212:Papilloma / Q000473:pathology