This 4 year old female patient presented with history of congenital erythroderma (without Colloidal membrane), which was evident during the first weeks of life and severe pruritic dermatosis. History of hair loss was given though it was not severe when she presented to me. Though lesions where predominantly ichthyosiform with a predilection for extremities, she also had eczematous peri-oral lesions characteristic of NS. However flexural lichenification was not prominent. ILC is a characteristic serpiginous migratory annular/polycyclic rash with double-edged scale.
More on the genomic and molecular aspects of this disease in my Applied – Bimatics (http://gulfdoctor.net/bioblog/2006/02/plasmin.html) Blog.