Keratoderma Hereditaria Mutilans (Vohwinkel's Syndrome): A Trial of Isotretinoin

An 8-year-old girl with the classic findings of keraloderma heredilaria mutilans (Vohwinkel's syndrome) was seen. Treatment with isotretinoin was instituted to decrease the hyperkeratosis and to prevent further autoamputation. After a 12-week course at 2 mg per kg per day, the patient had only minimal decrease in the amount of hyperkeratosis. Because of Ihe well-known long-term risks of systematic retinoids and her suboptimal improvement, therapy was discontinued.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/74902/1/j.1525-1470.1985.tb01056.x.pd