Darier-White syndrome

(redirected from follicular keratosis)

Darier-White syndrome

A rare autosomal dominant disorder of keratinisation characterised by multiple focal keratotic papules with acantholytic dyskeratosis. It first appears in childhood or adolescence with the formation of numerous flesh-colored papules in sebaceous areas of the skin glands—chest, scalp, forehead, back, ears, groin, nasolabial folds—which, with time, become encrusted. The nails are dystrophic, palms possibly pitted; variable retinitis pigmentosa and cataracts.

Molecular pathology
Defect in the desmosome-keratin filament complex due to ATP2A2 mutations.
References in periodicals archive ?
Age, y/Sex Site Slide 1 11 70/M Nose AK 29 75/M Ear Inverted follicular keratosis 63 27/F Nose Cyst 72 73/F Cheek AK 84 50/F Scalp SK, inflamed 95 19/M Face Lentigo 147 62/F Face AK 203 32/M Trunk Nevus 215 55/F Nose Fibrous papule 279 42/M Forearm Nevus 306 60/M Scrotum Epidermal hyperplasia 310 45/F Thigh Lentigo 395 69/M Shoulder AK 459 79/F Nose AK 464 83/M Ear AK Case No.