Paul Blum,1 in an article entitled "États ichtyosiformes," gave the following points as characteristic of congenital ichthyosiform erythroderma and as differentiating this clinical entity from ichthyosis:
The process as a rule is strictly congenital or appears soon after birth.
Hyperkeratosis is often generalized and is more accentuated than in ichthyosis.
A decided redness of the skin (erythroderma) is present. This sign, according to Darier, may not be constant, and for that reason he preferred the name hyperkératose ichtyosiforme instead of érythrodermie ichtyosiforme.
The process attacks the articular folds, the areas of predilection differing from those in the majority of cases of ichthyosis. Instead of sparing the folds, the lesions, on the contrary, find their greatest development here; the flexor surfaces and the median surfaces of the trunk are much more involved than, for instance, the lateral surfaces.
The lesions, instead of being anhidrotic, are often hyperhydrotic.
The histologic structure