clinical features

Last reviewed 01/2018

These include:

• brown follicular keratotic papules distributed over the seborrheic areas of the body - chest, back, ears, nasolabial folds, scalp and groin

• flat wartlike papules on the dorsa of the feet and hands

• punctate keratoses on the palms and soles - raised or with a central pit

• nails show longitudinal pink or white bands with characteristic V shaped notches at the free edges of the nail plates

• white centrally depressed papules of the mucosae of the cheeks, hard and soft papules, and gums

• exceptionally, permanent alopecia

Systemically, there may be:

• diminished intelligence
• chronic herpetic and bacterial infections secondary to a defect in delayed hypersensitivity

Reference:

• 1. Khachemoune A, Lockshin B.N. Chronic papules on the back and extremities. JFP 2004;53(5)
• 2. Szigeti R, Kellermayer R. Autosomal-Dominant Calcium ATPase Disorders. JID 2006;126:2370-2