DEFINITION.—Progressive unilateral facial atrophy is a disease characterized by progressive wasting of the skin, connective tissue, fat, bone, and more rarely muscles of one side of the face.
SYNONYMS.—Progressive facial hemiatrophy, Neurotic atrophy of the face (Virchow), Facial trophoneurosis (Romberg), Prosopodismorphia (Bergson), Atrophy of the connective tissue of the face or Laminar aplasia (Lande).
HISTORY AND LITERATURE.—This disease has been known to the medical profession since 1825, when, according to Eulenburg, it was first described by Parry. The Index Catalogue contains references to thirty books and articles on the subject in various languages. Eulenburg gives a bibliography which contains thirteen references not given in the Index Catalogue. One personal case, which will be detailed later, has fallen under observation. In all, I have collected from fifty to sixty cases. The number of reported cases is slowly increasing; nevertheless, the disease must still be regarded as rare. Carswell,1 according to Lasègue,2 first arranged scientifically the various lesions which might arise in consequence of a retardation and arrest, or even inverted action, in nutrition, and at the same time that he assigned their causes pointed out the several forms of atrophy. Aran3 has written a contribution on the subject, furnishing valuable material for classifying atrophies. Romberg called general attention to the disease in his clinical researches, published in 1846. He described the disease under the name of a new form of atrophy of the face, and took the position that it was a primary trophoneurosis. Of contributions by American writers, a paper by Bannister4 is the most complete and valuable. This not only contains two fully and carefully reported cases, but also a thorough discussion of special symptoms and the pathology of the disease. The chapter of Eulenburg5 contains an excellent sketch of the disease, and is especially valuable for the discussion of the various theories as to its nature.
1 Illustrations of the Elementary Forms of Disease, 1836.
2 Archives générales de Médecine, May, 1852, p. 71.
3 Ibid., Sept. and Oct., 1850.
4 Journal of Nervous and Mental Disease, vol. iii., No. 4, Oct., 1876, p. 539.
5 Ziemssen's Cyclopædia of the Practice of Medicine, vol. xiv., 1877.
ETIOLOGY.—The disease is of much more frequent occurrence among females than males. Out of 25 cases which I have been able to classify, 9 were in men and 16 in women. It is an affection of comparatively early life. The following were the ages of 25 patients: under ten years, 7; from ten to twenty years, 11; from twenty to thirty years, 6; over thirty years, 1.
Graefe6 reports a case due to syphilis, in which there was also paralysis of the left oculo-motor externus and trigeminus. In one of Bannister's cases the patient had suffered repeatedly from slight frost-bites. Among the causes which seem to be thoroughly established traumatism holds the first place. The affection could be traced to injury in a fair number of the published cases. A case is reported by Maragliano7 of a child who had a fall in which she struck the left half of her face, and in consequence of which a circumscribed swelling appeared near the external angle of the left eye and remained for a few days. Shortly after this the left half of the face began to grow smaller than the right. Schuchardt8 speaks of a fall on the head which left a cicatrix on the right parietal bone below the coronary suture, the injury being followed by facial atrophy. Panas9 reports a case in which the wasting of the face followed a fracture of the lower jaw. In one of two cases reported by Bannister,10 a man, aged forty-two, is stated to have been thrown from a stage-coach, falling on his head, and not losing consciousness, but receiving a scalp wound over the coronal suture. Subsequent to this time he had cerebral symptoms, physical and mental, and unilateral facial atrophy developed.