ABSTRACT

Zinsser-Engman-Cole syndrome A very rare condition in which the skin becomes thickened and pigmented and the nails are shed some time after 5 years of age. The skin may also be reddened and small areas of fine blood vessels (telangiectasia) may appear. The hair is sparse and the teeth irregular. White patches and ulceration of the tongue and lining of the mouth may lead to malignancy. The eyes may become affected. The bone marrow frequently reduces or stops producing blood cells and this (or malignancy) leads to death in childhood or early adult life. It is sometimes associated with mental handicap and delays in physical development including shrinkage of the testes. It nearly always occurs in males and is therefore thought to be caused by sex-linked inheritance.