title: High-Potency Topical Steroids: An Effective Therapy for Chronic Scalp Inflammation in Rapp-Hodgkin Ectodermal Dysplasia.
abstract: Chronic erosive pustular dermatitis with a predilection for the scalp is a hallmark of ectodermal dysplasias (EDs) caused by mutations in TP63, including Rapp-Hodgkin and Hay-Wells EDs. It is among the most troublesome and symptomatic complications and is typically refractory to classic wound care approaches. We report two cases of Rapp-Hodgkin ED with refractory scalp erosions that markedly improved with the use of potent topical steroids. We also note marked similarities between this scalp inflammation and "erosive pustular dermatosis of the scalp," a condition more typically found in elderly individuals with severe scalp sun damage, and speculate about possible shared pathogenetic mechanisms.
authors: Department of Pediatric Dermatology, University Children's Hospital Zurich, Zurich, Switzerland.|Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
authors: Martin
authors: M
authors: Theiler
authors: Department of Dermatology and Pediatrics, University of California, San Francisco, San Francisco, California.
authors: Ilona J
authors: IJ
authors: Frieden
mesh_terms: D000287:Administration, Topical; D002971:Cleft Lip / Q000150:complications / Q000188:drug therapy*; D002972:Cleft Palate / Q000150:complications / Q000188:drug therapy*; D004476:Ectodermal Dysplasia / Q000150:complications / Q000188:drug therapy*; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007249:Inflammation; D008297:Male; D012536:Scalp Dermatoses / Q000150:complications / Q000188:drug therapy*
chemical_list: D005938:Glucocorticoids