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.
Division of Oral Pathology and Oncology, University of Colorado School of Dentistry, Denver.
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Greer
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Reissner
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