ANSWER: APPLES – Test Question

The correct answer is A. Angioid streaks

Correct Answer Explanation

This photo depicts the plucked-chicken skin appearance of Pseudoxanthoma elasticum. Histologically, it presents with fragmented distorted calcified elastic fibers within the deep dermis. Fibers stain with von Kossa. It occurs due to a ABCC6 gene defect. Angioid streaks or rupture of Bruch’s membrane can occur with PXE.

Incorrect Answer Explanation

Lester sign, or hyperpigmentation of the pupillary margin of the iris, occurs in Nail-patella syndrome.

Lisch nodules, or iris hamartomas, occur in Neurofibromatosis type 1.

Glaucoma can occur in Neurofibromatosis type 1 and Sturge-Weber syndrome.

Congenital hypertrophy of retinal pigment epithelium can occur in Gardner syndrome.


Spitz JL. Genodermatoses: A Full-Colored Clinical Guide to Genetic Skin Disorders. Baltimore: Williams & Wilkins; 1996.