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Hemiatrophy of the Face

disease, atrophy, affected, muscles, portion and inflammatory

HEMIATROPHY OF THE FACE Hemiatrophy of the face is a progressive emaciation of one-half of the face including the skin, muscles and bones. The disease is not very rare in childhood, at least the beginning of the malady can in many cases be traced to that period of life. After the thirtieth year the disease according to Mobius does not occur. Girls arc more frequently at tacked than boys and the left side more frequently than the right. In rare cases both sides of the face are affected by the atrophy (as observed by the writer in a girl of seventeen).

The cause of the disease is unknown, as its nature has as yet never been properly explained. The slight knowledge we have of the pathology of the disease (Mendel, and especially Lobel and Wiesel) would seem to indicate an interstitial inflammatory process in the trigeminal nerve including the Gasserian ganglion as the original cause of the disease. According to this view hemiatrophy of the face is therefore a chronic inflammatory disease of the peripheral portions of the trigeminus. It is possible that inflammatory processes in the various portions of the head (erysipelas, angina and the like), mention of which is some times found in the history- as forerunners of the disease, really have some etiologic significance. In other cases there is a possibility of toxic or infectious substances having invaded the tissues through the tonsils and produced a hemiatrophy of the face (illobius).

The disease begins with atrophy of a limited portion of the skin of the face, which becomes attenuated and loses its subcutaneous fat, so that it can be taken up in minute folds. Sometimes brownish discolora tion of a small portion of the skin is observed. These changes usually begin in the cheek, in the canine fossa. The atrophy rapidly spreads to the muscles and bones, causing depression especially of the zygoma and of the upper jaw. The atrophy ultimately effects the entire half of the face, which presents a sharp contrast to the healthy side, particu larly along the median line, the forehead, lips and chin. The line of

separation is sometimes convex toward the sound side, as though the latter were endeavoring to surround the diseased half. The tongue, the upper and lower jaws, and the pharyngeal structures share in the hemiatrophy in severe cases, and the hair falls out on the affected side of the scalp. Neuralgic pains are sometimes complained of at the beginning of the disease as toothache. Paralysis of the muscles of the face is entirely absent and, although the muscles of mastication share in the general emaciation, they present no functional weakness.

The course of hemiatrophy of the face is progressive in so far as in the majority of the cases the entire half of the face is ultimately affected. The disease then becomes arrested and it is even said that in some cases the diseased half of the face fills out again. In sonie instances the atrophy is confined to a small portion of the cheek.

The diagnosis is clear at the first glance. The only possible source of error is an old facial palsy with secondary asymmetry- of the face sim ulating hcmiatrophy. In fact, Frombold-Treu in his monograph of this disease mentions a large nuniber of alleged cases of hemiatrophy which do not belong to t he group at all.

Tbe treatment of circumscribed hemiatrophy of the face is powerless and we therefore hail with joy the recent efforts of Gersuny and Moskowicz to correct the deformity by repeated subcutaneous injections of paraf fine in the affected area. The result is remarkably good, and as the dis ease, while painless, is nevertheless greatly disabling on account of the striking change in the expression of the face, the results so far as the patient is concerned are equivalent to an actual cure of the disease.