INTRODUCTION
Ectodermal dysplasias (ED) are a heterogeneous group of disorders
resulting from abnormalities of structures derived from embryonic
ectoderm. Rapp-Hodgkin syndrome (RHS) was first described over 30 years
ago in an affected mother, son, and daughter with a combination of ED,
cleft lip and cleft palate (1). Other features include narrow nose, thin
coarse hairs, hypodontia with hypoplastic enamel and malformed teeth,
hyponychia, anonychia, narrow or dystrophic nails, lacrimal duct
abnormalities, ear and ear canal abnormalities (2). Here, we describe a
15-year-old male who has RHS associated with palmoplantar keratoderma.