DERKUMA DISEASE (F. X. Dercum, amer. neuropathologist and psychiatrist, 1856 — 1931; synonym: Derkum's syndrome, painful lipomatoz, lipomatosis dolorosa) — the neuroendocrinal disease, characteristic feature to-rogo is education in hypodermic cellulose of multiple painful lipomas or the diffusion localized fatty educations. It is for the first time described in 1888.
The disease meets rather seldom, affects preferential women in the postclimacteric period, very seldom men.
The etiology and a pathogeny
the Aetiology and a pathogeny are not established. Communication of a disease with primary defeat of hypothalamic area since in the anamnesis of patients instructions on a craniocereberal injury or an infectious disease of c are frequent is supposed. N of page (encephalitis, meningitis). In favor of such assumption also frequent combination testifies Must be with psychological disturbances and the polyglandular nature of defeat of endocrine system (see. Polyglandular insufficiency ). From this point of view development of a lipomatoz is a consequence of disturbance of neurohumoral regulation of a lipometabolism, i.e. only one of symptoms of the general disease. Primary role of hormonal frustration in connection with a menopause, and also allergies is not excluded. The assumption of hereditary character Must be is based on observations of a disease at several members of one family.
The pathological anatomy
the Most characteristic is a structure of fatty tumors: they can be various in size, a form and a consistence, but differ in abundance of vessels with the expressed perivascular lymphoid infiltration and development of connecting fabric (angiofibrolipoma) from usual lipomas. Also degenerative and dystrophic changes of the majority of closed glands in the form of their sclerosis, an atrophy, cystous regeneration are characteristic; the most expressed changes are noted in a hypophysis, a thyroid gland, an adrenal gland. Changes from other bodies and fabrics (e.g., osteoporosis, dystrophic changes of sympathetic nodes, etc.) are less characteristic and have, apparently, secondary character.
The clinical picture
the Disease develops slowly and only gradually gets characteristic a wedge, a picture. The first symptoms often are the accruing adynamy, muscular weakness, pain in various parts of a body. Mental disturbances can be expressed in various degree, to a thicket reactive depressions are observed? decrease in memory, morbid depression, sometimes develops dementia. The neurotic phenomena (headaches, dizzinesses, paresthesias), trophic disturbances (a hair loss, fragility of nails, disturbance of sweating, etc.), a hypothyroidism are frequent.
The basic a wedge, display of a disease — patol, fatty deposits on which nature distinguish nodal, the diffusion localized and diffusion and extended forms of a disease.
Nodal form it is characterized by education in a hypodermic fatty tissue of multiple fatty nodes which size varies from 0,5 to 10 cm. From usual lipomas these educations differ, in addition to large number, in a little bigger density, quite accurate otgranichennost from surrounding cellulose and morbidity at a palpation. Degree of morbidity can be various — from insignificant to very expressed. Localization of nodes is various, usually asymmetrical; never hands and feet are surprised. Especially often nodes are located on forearms (fig. 1), shoulders, hips, a trunk, is very rare on a face. At long existence they become more dense owing to development of connecting fabric and can be accustomed to drinking with skin and the subject fabrics.
At to the diffusion localized form there are large sites of adjournment of subcutaneous fat separated from each other by the furrows which are formed in areas with normal hypodermic cellulose. Characteristic localization of these fatty deposits — a chest wall, a stomach, hips, especially on their inner surface, over knee joints. In certain cases a local adiposity, stretching skin, can reach the big sizes, forming the thick fold which is hanging down in the form of an apron (fig. 2). Skin over these fatty educations is not changed a thicket, at long existence of a disease can pererastyagivatsya and be accustomed to drinking with patol, deposits of subcutaneous fat.
Diffusion common form it is characterized by a combination of the general symptoms Must be with the obesity which was more expressed in the lower half of a body. Also the mixed forms of a disease meet.
the Diagnosis is established on the basis of the characteristic painful fatty deposits which are combined with an adynamy and psychological disturbances.
The disease differs from a usual lipoma in plurality of educations, morbidity of nodes, existence of psychological and endocrine disturbances. At a neurofibromatosis nodes have more dense consistence, the smaller size, are often localized in a projection of nervous trunks, there are no mental and endocrine disturbances.
Treatment owing to lack of etiotropic and pathogenetic methods has symptomatic character and is usually ineffective.
At symptoms of hypofunction of separate closed glands the corresponding endocrine drugs are used (steroid hormones, Thyreoidinum, are oestrogenic). The diet interfering progressing of obesity is appointed, and at psychological disturbances — psikhofarmakol, means.
Surgical removal of separate nodes is inefficient owing to their usual recuring near the place of operation or in other areas of a body. Nevertheless it can be shown at the sharp morbidity of nodes interfering function of this or that joint or wearing clothes.
the Forecast concerning life usually favorable, a disease can slowly progress for decades. Concerning recovery the forecast is adverse; with development of a disease psychological disturbances progress. Owing to existence of polyglandular endocrine insufficiency, including and adrenal, big operative measures at patients Must be, made without the corresponding preparation, can pose hazard to life of the patient.
Bibliography: V. G rams. Diseases of endocrine system and metabolism, L., 1955; Gadzhiyev of X. AA., Shamov I. A. and Magomedov of III. M. O of Derkum's disease, Klin, medical, t. 48, N ° 7, page 133, 1970; Ignatyev A. S. and And in and N about in and I. A. O of Derkum's disease, Vestn, hir., t. 102, No. 6, page 123, 1969; The Guide to endocrinology, under the editorship of B. V. Alyoshin, etc., page 84, M., 1973; Sh and m about in I. A. Hereditary form of a disease of Derkum, Klien, medical, t. 54, No. 6, page 119, 1976; D e with u m F. X. A subcutaneous connective tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema, Univ. med. Magaz., v. 1, p. 140, 1888; de GennesL., Bricaire et DuruptL. Syndrome de Dercum, Bull. Soc. med. Hop. Paris, t. 62, p. 337, 1946; K o g 1 e r A. t)ber psychische Storungen bei Adipositas dolorosa (Morbus Dercum), Nervenarzt, Bd 14, S. 18, 1941.
B. S. Pomelov.