LEUCONYCHIE TOTALE ET DYSPLASIE ECTODERMIQUE. OBSERVATION DE DEUX CAS

The authors describe two brothers presenting leukonychia totalis in association with deafness, hyperkeratosis palmaris and plantaris, scalp modifications and dental lesions. The onychopathia began during early childhood and was also present in four other members of the same family. All nails of both hands and feet were affected and appeared white, dull and with inconspicuous lunulae. The hair was rough, dark, bristly, looking like steel wire. In particular, changes of the hair shaft were studied under sanning electron microscope. Hair dysplasia, accompanied by structural changes such as complete and incomplete twisting, longitudinal grooves and modified imbrication, was found. After briefly reviewing the literature on the subject, the authors point out the suggestive hypothesis that all these manifestations might possibly be ascribed to a single genetic defect, in spite of their different clinical features in various patients.