The first successful treatment of two severe congenital skin diseases has been reported by Edmund Klein, MD, associate chief of the Department of Dermatology at Roswell Park Memorial Institute, Buffalo.
While both diseases are rare, Klein suggests that the method used—administration of extremely low doses of antimitotic agents—may present a new therapeutic approach to proliferative skin diseases, possibly including some forms of cancer.
The two genetic diseases are ichythyosis linearis circumflexa (ILC) and poikiloderma congenitale (PC). ILC involves erythematous or "scaly" exfoliations over the whole body surface as does PC. In addition, however, persons with PC have decreased function of apocrine and eccrine glands, reduced pigmentation, telangiectasia leading to angiomas and, in the advanced stages of the disease, ophthalmic and bone problems.
The man with ILC received an initial 50-mg dose of cyclophosphamide and three weeks later began to receive 50 mg every three days, which was subsequently reduced to