Osteopathia striata and focal dermal hypoplasia

A 43-year old female, presenting with pain in the left knee, attributed to a stress fracture of the tibia, was found to have osteopathia striata and numerous other congenital anomalies, not only of the skeleton, but also of other tissue systems. Of the latter the most important was the presence of focal dermal hypoplasia, a disorder of the skin. Review of the radiological and dermatological literature has shown this to be the fourteenth case in which these excessively rare entities have been associated. Such an association is considered to be more than a coincidence. A radiological diagnosis of osteopathia striata, therefore, should be followed by a careful dermatological examination for focal dermal hypoplasia. Conversely, diagnosis of this rare dermatological disease should indicate the necessity for radiological survey of the skeleton.