Pop Quiz #199

The correct answer is A. Ectodermal dysplasias.

Ectodermal dysplasias. The picture demonstrates incontinentia pigmenti. Both ectodermal dysplasias (e.g. hypohidrotic ED) and incontinentia pigmenti have pegged teeth.

Pachyonychia congenita type 2 has natal teeth. 1

Retention of primary teeth can be seen in Hyper-IgE syndrome. 1

Gardner syndrome has supernumerary teeth (polydontia). 1

Sjogren-Larsson syndrome, tuberous sclerosis, and junctional epidermolysis bullosa has enamel hypoplasia (including enamel pits). 1

Papillon-Lefevre and Haim-Munk syndrome have periodontitis with early tooth loss. 1

Letterer-Siwe syndrome has floating teeth. 1

Congenital erythropoietic porphyria has red-colored teeth (erythrodontia). 1


1. Jain S. Dermatology – Illustrated Study Guide and Comprehensive Board Review. Springer International Publish; 2017.