Lipodystrophy

LIPODYSTROPHIA, a morbid form first described in 1911 by Simons under the name lipodystrophia progressiva and characterized by a peculiar distribution of subcutaneous fat: atrophy of the subcutaneous fat layer on the upper half of the body with its hypertrophy in the region of the pelvis and lower extremities. If the difference between the circumference at the level of the chest and the pelvis is normally on average 11% (O. V. Meyer), in lipodystrophy it averages 40–50%. Lately, a number of forms diverging more or less sharply from the classical forms of Simons have begun to be referred to lipodystrophy (Geymanovich and others); it is pointed out, among other things, that this disease is an exaggerated expression of the fat distribution pattern characteristic even of healthy women (especially with an asthenic body type). Sometimes in the initial periods of Basedow's disease, this kind of fat distribution is observed, albeit in a milder form. Pathognomonic in the clinical picture of lipodystrophy is the atrophy of the upper half of the body, in particular the sharp emaciation of the face, which acquires the appearance of a "dead mask." In places where fat disappears, the skin nevertheless remains elastic and is not atrophic. The accumulation of fat in the upper half of the body cannot be achieved under any conditions of forced nutrition. Psychically, patients are normal; the indication that lipodystrophy is accompanied by depression has no general significance. Only about 100 cases have been described, including atypical ones. The disease in 80% of cases begins in the first ten years of life and occurs almost exclusively in women. The course of the disease is for the most part progressive only in the initial period; later, a stable state ensues, and therefore the name "lipodystrophia progressiva" does not characterize it entirely accurately; it would be more correct to speak simply of lipodystrophy. The etiology is completely unclear. There are vague indications of a hereditary-constitutional factor (Bauer, Wilder); postencephalitic lipodystrophy has been described. The pathogenesis is also unclear. Some authors associate lipodystrophy with an endocrine disorder, primarily with hyperthyroidism (Marapon), with a disturbance of pituitary function (Marburg, Evzerova). Disturbances of fat metabolism as a rule are not observed. Sereisky is inclined to associate lipodystrophy with autonomic (diencephalic) insufficiency, wherein the disorder of endocrine gland activity acts as a sort of activator. Thus, a disturbance in the function of the endocrine glands, which are also affected during pregnancy, can serve as a trigger for the formation and development of lipodystrophy. To elucidate the pathogenesis of lipodystrophy, observations by Depisch are of undoubted interest; he succeeded in inducing local lipodystrophy at the injection sites by means of prolonged insulin injections.