Two brothers with keratosis follicularis spinulosa decalvans

Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder affecting both, the skin and eyes. There are few reports about this entity. The aim of this report is to describe 2 brothers with progressive scarring alopecia of the scalp, hypotrichosis with follicular prominence of the eyelashes, and extensive keratosis pilaris. The second-patient has Down syndrome with palmoplantar keratoderma and partial alopecia of the eyebrows. We also reviewed the literature about this uncommon entity.