Clinical features

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

References:

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.