ERITROMELALGYYa (erythromelal-gia; Greek erythros red + melos extremity + algos pain; synonym: S. W. Mitchell's syndrome, a disease Weir-Mitchell) — the form of an angiotrofonevroz which is characterized by attacks of sudden thermalgias preferential in distal departments of extremities with a bright local hyperemia and a cutaneous dropsy.
It is for the first time described in 1872 by S. W. Mitchell. Distinguish an eritromelalgiya as an independent disease (primary E.) and as the syndrome developing against the background of various patol. processes (secondary E.).
Reason primary E. it is not established. Secondary E. the nek-eye accompanies neurologic diseases (a myelitis, a myopathy, poliomyelitis, multiple sclerosis, a myelosyringosis, back tabes, a truncitis, etc.); to intoxications (alcoholic, mercury); to endocrinopathies (hypothyroidism, hypocorticoidism, diabetes mellitus); to diseases of blood (polycythemia); nek-eye to defeats of vascular system (obliterating defeats of vessels of extremities); to the metabolic disturbances connected with growth of a malignant tumor; to vertebrogenny angiotro-fichesky syndromes, etc. To development E. frequent and long overheatings or overcoolings of extremities, bystry and sharp change of ambient temperature, wearing close footwear promote.
The pathogeny is studied insufficiently. Consider that pristupoobrazno the developing local hyperemia and a cutaneous dropsy have neurogenic character. At the same time plays an essential role patol. a condition of receptor systems of vessels of the struck area, (see Receptors), disturbance of microcirculation (see), a fabric blood-groove and transcapillary exchange. It is established that during an eritrome-lalgichesky attack (crisis) about -
local reorganization of a fabric blood-groove due to increase in arterial inflow of blood through shuntovy microvessels proceeds (arteriolo-venules yarny, arterio a loarteria -
330 ERYTHREMIC MYELOSIS
a ny anastomosis). Expansion of an anastomosis and change of a blood-groove cause irritation of the terminations of sympathetic nerve fibrils in walls of intraorganic vessels and cause developing of pains like simpatalgiya.
AA. it is observed equally often at women and at men. Usually E. arises aged from 20 up to 50 years; occurs at children seldom. The Eritro-melalgichesky attack arises suddenly. There is a thermalgia in symmetric sites of extremities (fingers, brushes, feet), usually along with both parties. In some cases emergence of pain in a nose, auricles, generative organs, mammary glands is noted. Pain amplifies during the warming of a body, in steep position of an extremity, and also at a touch and pressure in the field of the painful site, napr, footwear, a blanket. During an eritromelalgichesky attack skin of the struck part of a body gains bright red color, its temperature in comparison with adjacent sites increases, there is a cutaneous dropsy; the site of a hyperemia and hypostasis usually has accurate outlines. Duration of attacks is various — of several minutes till several o'clock. The course of a disease chronic, quite often progressing. At the same time attacks become frequent, their intensity and duration increase. Over time zone patol. changes can extend, in the struck departments of extremities trophic changes develop — skin becomes cyanotic, dry, edematous, nails fragile, sometimes there are ulcerations of skin. In the mezhpristupny period acroparesthesias can be observed. At nevrol. inspection reveal a hyperesthesia or a hyperpathia (a perversion of sensitivity) in distal departments of extremities (see Sensitivity). The condition of patients especially worsens in hot season.
Pristupoobrazny development of characteristic symptoms allows to establish the diagnosis of an eritromelalgiya already at its early stages. Specification of the diagnosis is promoted by the results received by means of a termografiya (see), kapillyaroskopiya (see), pletizmografiya (see) to-rye allow to reveal the local disturbances of microcirculation inherent to angiotrofonevroza (see).
The differential diagnosis is carried out from akroeritroza? at to-rykh, unlike E., the dermahemia of distal departments of extremities is not followed by simpatalgiya. A current of akroeritroz more favorable and at elimination of provocative factors (stay in the crude, cold room, freezing injury etc.) perhaps spontaneous recovery. In differential diagnosis primary and secondary E. it is necessary to consider that at the last symptoms are usually less expressed, defeat is more often unilateral, a current more high-quality. Besides, at secondary E. treatment of a basic disease leads to subsiding of the phenomena of an eritromelalgiya.
Treatment complex. During an eritromelalgichesky attack apply vasoconstrictors (a phenylephine hydrochloride, ephedrine); serotonin and serotoninopodobny drugs (Peritolum, Mexaminum); antihistaminic drugs (Suprastinum, tavegil); the means influencing on fabric exchange (ascorbic to - that, Rutinum, Venorutonum); drugs of calcium (calcium gluconate, Calcii chloridum). In the mezhpristupny period conduct courses of reflexotherapy (see), including the acupuncture (see) tempering and physiotherapeutic procedures (two - or four-chamber bathtubs from acetilsalicylic to - that, carbonic bathtubs). In hard cases sometimes resort to novocainic and perineural (futlyarny) blockade of large nervous trunks of extremities, intramuscular administration of corticotropin. At secondary E. carry out treatment of a basic disease.
The forecast is, as a rule, more favorable at secondary E. Pervichnaya E. usually hardly will respond to treatment. Cases of spontaneous recovery are described, however generally the disease lasts is long with gradual decrease in working ability of patients.
Prevention consists in the elimination of unfavorable conditions of work and life promoting development E.
See also Angiotrofonevroza. Bibliogralekseev P. P. and Kost yu to A. N. O a pathogeny, clinic and treatment of an eritromelalgiya of Viyer Mitchell, Works Smolensk, medical in-that, t. 43, page 29, 1974; Diseases of a nervous system, under the editorship of P. V. Melnichuk, M., 1982; In e y A. M., Solovyov A. D. and Kolosov O. A. N. Vegeto-vascular dystonia, M., 1980; Mitchell S.
W. Clinical lecture on certain painful affections of the feet, Philad, med. Times, v. 3. p. 81, 113, 1872. _ E. I. Ming of the cop.