CONGENITAL ichthyosiform erythroderma was described by Brocq 1 in 1902 after the prolonged observation of several patients in whom the disease was characterized by diffuse redness beginning at birth, a shiny appearance of the face and widespread hyperkeratosis resembling ichthyosis. The first and most important consideration of the subject in the English literature was by MacKee and Rosen, 2 whose important monograph was published in 1917. These authors completely reviewed the literature on the subject and presented the history of the disease and a detailed discussion of the clinical and histologic features. MacKee and Rosen also discussed the nosologic position of congenital ichthyosiform erythroderma, especially regarding its relationship to ordinary ichthyosis. These authors found that up to 1917 a total of 45 cases of the disease had been reported. In 1925 Weiss and Tobias 3 reviewed the literature from 1917. They found 10 additional cases and reported 6 of their own.