Patients with ichthyosis, follicular atrophoderma, hypotrichosis, and hypohidrosis present a normal stature, diffuse congenital ichthyosis, patchy follicular atrophoderma, generalized and diffuse non-scarring hypotrichosis, and marked hypohidrosis. Steroid sulfatase activity is normal. Electron microscopic studies of ichthyotic skin do not show any specific abnormality. Subjects with ichthyosis, follicular atrophoderma, hypotrichosis, and hypohidrosis are usually reminiscent of Bazex syndrome in which ichthyosis is not a component.