Also known as keratosis follicularis.
A keratinocyte adhesion disease with grouped keratotic papules aggravated by heat and sweat, with the tendency of developing the third and fourth decades of life.
Autosomal dominant disorder, caused by germline mutations in the ATP2A2 gene, which causes haploinsufficiency of epidermal calcium ATPase.
It affects the body symmetrically, but lesions can be arranged along the lines of Blaschko in segmental disease.
Skin findings are scaling papules on face, trunk, and flexural areas with roughness.
Onset childhood with slow progression over lifetime.
Treated with oral and topical retinoids.