Rapp Hodgkin Syndrome

© 2017 Indian Dermatology Online Journal | Published by Wolters Kluwer Medknow Ectodermal dysplasias are a heterogeneous group of disorders resulting from abnormalities of structures derived from embryonic ectoderm (skin, teeth, and appendage). Rapp Hodgkin Syndrome (RHS) is a type of anhidrotic ectodermal dysplasia, which was described by Rapp and Hodgkin in 1968.[1] This syndrome is characterized by anhidrotic ectodermal dysplasia, cleft lip, and cleft palate. Other features, which have been described by various authors include coarse and wiry hairs, small mouth, narrow nose, oligodontia or anodontia, conical teeth, anonychia, hyponychia, narrow or dystrophic nails, lacrimal duct abnormalities, ear and ear canal abnormalities, and genitourinary abnormalities.[2,3] It has autosomal dominant inheritance. Here, we report a rare sporadic case of RHS with characteristic manifestations.