It is inherited as an autosomal dominant character and thus, some other member of the family, especially one of the parents at least, should also manifest the disease. The lesions are usually not present at birth, but develop during the first year of life.
These consist of dirty looking, brownish, angulated scales which are present all over the body but are most prominent on the anterior surfaces of the legs. Flexural surfaces of the joints such as the cubital and the popliteal fosae, axillae, groins and other skin folds are as a rule, not involved.
The face and the scalp are also not involved. Some patients in addition, have a diffuse hyperkeratosis of the palms and the soles and occasionally, they may also have small asymptomatic micro-papules around the hair follicles. [Read more...]
This is an uncommon but severe variant of lamellar Ichthyosis. The child at birth is encased in a thick sheet of hyperkeratosis which shows deep fissure at the folds of skin.
