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areas originally whitish subsequently acquire a brownish hue. In these situations the skin is extremely thin, is wrinkled like a cicatrix, and the layer of subcutaneous fat particularly is found to be greatly wasted. Cutaneous sensibility is not rarely found to be impaired. These alterations result in great disfigurement, as the affected side of the face appears to be shrunken and the seat of cicatricial contraction (Fig. 19). The deformity becomes the more marked if the bones of the face also are involved in the atrophic process. Particularly the superior and the inferior maxilla and the malar bone frequently appear atrophied upon one

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FIG. 19.-Appearance of the face in a case of left-sided progressive facial atrophy.

side, and are conspicuous for their sharp bony processes. In the jaws the absence of some of the teeth has been observed. Excision and examination of the muscles of the face have disclosed that these also may be involved in the atrophic process. In any event, unilateral atrophy of the tongue and of the muscles of the soft palate not rarely occurs. The cartilages of the nose and the car also are at times involved in the atrophic process. The eye upon the affected side is frequently retracted into the orbit, because the retrobulbar fatty connective tissue has disappeared, and the palpebral fissure appears at times contracted, at other times dilated. The nasal and buccal orifices also not rarely exhibit unilateral

dilatation. Occasionally symptoms of unilateral sympathetic irritation (pallor of the skin of the face, dilatation of the pupils, and unilateral anidrosis) are observed. The disorder pursues a course of variable rapidity, and is followed by permanent disfigurement of the face. In one case the trigeminal nerve upon the affected side was found to be the seat of chronic proliferating neuritis, although the observation is so unique that it can scarcely be utilized in explanation of the disorder. Experience has shown that the affection occurs most commonly in men, and it generally sets in between the tenth and the fifteenth year of life. It begins but seldom after the twenty-fifth year. The patients are often members of neurotic families, and occasionally have previously suffered from trigeminal neuralgia, spasm of the facial muscles, hemicrania, or epilepsy. Unilateral atrophy of the face has been observed also in cases of tabes dorsalis and multiple cerebrospinal sclerosis. Exposure to cold, traumatism, and infectious disease also are mentioned as causative factors. The disorder is not dangerous, but incurable. Confusion with congenital asymmetry of the face is scarcely conceivable, as the condition under consideration is not a congenital one.

FACIAL HEMIHYPERTROPHY.

Unilateral hypertrophy of the face is a rare disorder, which occurs only as a congenital condition, and occurs with equal frequency in both sexes and upon both

sides of the face. The soft parts of the face especially are involved in the hypertrophic process, but the tongue and the tonsil also have been hypertrophied on one side. Not rarely the arm and the leg also upon the same side of the body are involved in the hypertrophic process. At times the hypertrophied half of the face is the seat of abnormal redness, of dark discoloration of the skin, and of increased growth of dark hair. The sebaceous secretion often is increased, and it often collects upon the skin in small crusts. The disorder is incurable, and causes disfigurement of the face (Fig. 20), but it is unattended with danger to life.

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FIG. 20.-Left-sided facial hypertrophy in a girl 9 years old (after Schieck)

SCLERODERMA.

Symptoms, Diagnosis, and Prognosis.-Scleroderma is a rare disorder attended with induration and contraction of the diseased portions of the skin. Generally it appears first in the form of irregular spots and bands, which gradually increase in size and coalesce, and finally transform large areas of skin into firm and hard tissue. The changes occasionally develop insidiously, and often are discovered accidentally on palpating the skin or in consequence of alteration in the expression of the face or of interference with the mobility of the extremities; or the induration of the skin may be preceded by swelling and redness, and this has been designated the edematous stage. The diseased portions of skin exhibit thickening and condensation, so that their boundaries can be readily determined by passing the finger over them. The skin cannot be easily raised in folds, and is but slightly movable upon the subjacent tissues. The color of the skin is at times unaltered, at other times remarkably pale, and at still other times excessively pigmented. Occasionally an abundant deposition of pigment has taken place at the periphery of the spots. The sudoriferous and the sebaceous secretion often exhibits no change. Cutaneous sensibility also is preserved. The electric resistance of the skin has been found at times unaltered, at other times increased or diminished. The indurated portions of skin frequently become thinned and undergo cicatricial contraction, and various deformities and functional disturbances result. Extensive scleroderma of the skin of the face gives the facial expression a mask-like rigidity, causes impaired mobility and narrowing of the palpebral fissure, and often also the formation of fissures and contraction of the buccal orifice, so that the ingestion of food is seriously interfered with.

Linear and annular seleroderma of the skin of the chest causes a sense of constriction and interference with breathing. Scleroderma of the extremities often interferes with the mobility of the joints and gives rise to permanent deformities of the fingers, hands, and arms. Occasionally the fingers are involved earliest and in greatest degree, and often these alterations are preceded by the manifestations of a vasomotor neurosis and erythromelalgia. Under such conditions the individual phalanges become shortened, particularly the terminal phalanges, and some of the nails are exfoliated, and even the entire terminal phalanx. This condition has been designated sclerodactyly (Fig. 21). Often ulcers resistant to treatment appear upon the fingers. In some instances sclerotic changes have been observed also upon some of the mucous membranes, such as that of the mouth, the pharynx, the larynx, and the vagina.

Seleroderma generally pursues a chronic course, but not rarely exhibits numerous variations, the sclerodermatous areas becoming

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again soft and healthy. In general there is an undeniable tendency for the changes in the skin to extend progressively. The patients become exhausted and die as a result of marasmus. prognosis, therefore, is not favorable, although there is no immediate danger to life. It may be mentioned, further, that occasional osseous and muscular atrophy and muscular sclerosis result in consequence of the compression exerted by the indurated and contracting skin.

Anatomic Alterations.-Microscopic examination of diseased portions of skin has disclosed increase and condensation of the connective tissue in the corium and in the subcutaneous connective tissue, thickening of the walls of the blood-vessels, and accumulation of round cells in the vicinity of the blood-vessels.

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FIG. 21. Sclerodactyly in a woman, 49 years old: from a photograph (personal observation, Zurich clinic).

Attention has recently been directed in several instances to atrophy of the thyroid gland, whence the theory has arisen that seleroderma is the result of an auto-intoxication originating from the thyroid gland, and that the disease is therefore genetically related to myxedema, exophthalmic goiter, akromegaly, and possibly also to unilateral atrophy of the face.

Etiology.-Scleroderma generally develops after the twentyfifth year of life, and occurs more frequently in women than in men. Exposure to cold, traumatism, emotional disturbances, and antecedent erysipelas have been mentioned among the causative factors. Occasionally the disease develops in the course of exophthalmic goiter, unilateral atrophy of the face, and Addison's disease. Treatment.-Among internal remedies, successful results at

times follow the administration of sodium salicylate (1.0-15 grains four times daily). Among external remedies, massage, baths, galvanization, and inunctions of oil applied to the diseased skin may be recommended.

MYXEDEMA.

Etiology. Three varieties of myxedema must be distinguished, namely, the spontaneous, the operative, and the congenital. Spontaneous myxedema occurs most often in women, and generally develops after sexual maturity, but rarely in childhood or after the fiftieth year of life. The patients often are neurotic individuals or members of neurotic families. In isolated instances heredity was present; in others, a familial distribution-that is, the occurrence of the disease in several brothers and sisters. Exposure to cold, concussion, antecedent infectious disease, emotional disturbances, especially, however, profuse menstruation, pregnancy, parturition, and lactation are believed to be causes of the disorder. Operative myxedema has been observed after total, and occasionally also after partial, extirpation of the thyroid gland. The designation cachexia strumipriva or thyreopriva also has been employed. Congenital myxedema, finally, occurs in association with dwarfism and idiocy, and is known also as sporadic cretinism.

Symptoms and Diagnosis.-The principal symptoms of myxedema consist in thickening of the skin, progressive cachexia, and nervous disturbances. In cases of spontaneous myxedema the thickening of the skin is generally appreciated earliest in the face, which presents a peculiar disfigurement. The eyelids are converted into thick sacs and the eyes appear half closed. The nose becomes enlarged and the lips everted. The facial expression becomes stupid and dull (Fig. 22). At the same time the skin is pallid and alabaster-colored, and feels cool. The sebaceous and sudoriferous secretions are diminished, and often desquamation of the skin takes place. The hair falls out as well as the teeth. Gradually the thickening of the skin extends also to the trunk and the extremities. Of the latter the legs are generally involved earliest, and especially the toes are transformed into plump structures suggestive of the appearance of the toes of the pachydermata. It is noteworthy that, in contradistinction from ordinary edema, pressure with the finger leaves no depression in the skin.· The gait of the patient is generally clumsy and waddling. Often the tongue becomes enlarged and speech slow and awkward. The bodily temperature is not rarely subnormal and the pulse slow. Some patients complain of paresthesia. Occasionally thickening. of some of the mucous membranes (mouth, intestine, larynx) occurs, and when the larynx is involved the voice becomes markedly deepened. In diagnosis it is important to find the

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