PSEUDOXANTHOMA ELASTIC (pseudoxanthoma elasticum; grech, pseudes false + xanthoma; synonym: elastic xanthoma of Balzer, elastic nevus of Gutmann, elastic pseudoxanthoma to Darya, elastoma of skin of Yuliusberg) — the disease, to-rogo is the cornerstone the hereditary dystrophy of elastic fabric which is shown in the form of the papular and atrophic centers of yellowish color which are localized on skin and mucous membranes. Patol, process at P. often gains system character, extending to a retina of eyes and blood vessels. Offered the name «pseudoxanthoma elasticum» in 1896 to Zh. Darya. Item e. it can be combined with Elers's syndrome — Danlosa and cutis laxa (see. Dystrophy of skin ).
Etiology and pathogeny
P. e. — a hereditary disease with an autosomal and recessive and autosomal and dominant (irregular) mode of inheritance. It is not excluded that a certain role in P.'s pathogeny e. play endocrine disturbances, and also various provocative factors (inf. diseases, injuries).
In a middle and deep part of a derma, sometimes in hypodermic cellulose, is observed a hyperplasia of elastic fabric, fibers cover bazofilna, are broken off, twisted in the form of lumps (elastorrhexis); their treatment by salts of calcium is possible. Such picture can be revealed both in sites of defeat, and in skin healthy by sight.
In cases, when P. e. gains system character, in addition to changes of skin, the damage of a retina of eyes consisting in fracturing in an elastic cover of a retina — Bruch's membrane is noted (a syndrome Grenblad — Strandberg).
System elastoreksis (the systematized elastoreksis Touraine, system elastosis of Mashkilleyson), in addition to skin defeats, includes the vascular changes resulting from a rupture of elastic fibers of a vascular wall and its calcification that leads to reduction of a gleam of vessels.
The disease is shown at young age, sometimes right after the birth. Favourite localization patol, the centers on skin — side surfaces of a neck, axillary and inguinal areas, elbow bends, popliteal spaces, generative organs, is more rare — a trunk. Face skin, palms and soles usually is not involved in process. Skin in the centers of defeat has a softish consistence, is slightly raised over the level of not changed skin, sometimes looks corrugated. On this background the flat, hardly towering, as if interspersed, soft small knots are visible to dia. from 1 to 5 mm. A peculiar color (yellowish as at old ivory) gives them looking alike xanthomas (see. Xanthoma ). Small knots are located linearly, chains, quite often form reticulation (tsvetn fig. 6), sometimes merge in continuous plaques with teleangiectasias and hemorrhages. Gradually affected skin becomes thinner, becomes flabby, «old». The skin collected pleated finishes very slowly. Sometimes on a stomach atrophic strips appear (see the Atrophy of skin). On a mucous membrane of a mouth, a vagina, a rectum, etc. wax-like-yellowish papular elements and spotty teleangiectasias are observed.
At a syndrome Grenblad — Strandberg on an eyeground the cracks reminding branchings of blood vessels (angioidny strips) are visible. The accompanying hemorrhages and hems which are formed on site cracks cause decrease in sight up to a total blindness.
System elastoreksis it is clinically shown by a hematemesis, nasal, intestinal and urogenital bleedings, hemorrhages in joints, a brain, reduction of pulse wave, lack of pulse on extremities, the alternating lameness. Elastoreksis leads to development of an aortitis, aortic aneurysm, early arteriosclerosis, arterial hypertension, stenocardia, myocardial infarction, cordial weakness at young age. Radiological calcification of walls of large arteries, including an aorta is defined.
Diagnosis put on the basis a wedge, pictures of existence of the typical centers of defeat and (in doubtful cases) data gistol, researches. Differential diagnosis is carried out with an atrophy of skin of other etiology (see. Atrophy of skin ) and a xanthomatosis (see).
Treatment. Effective methods of treatment are absent. Apply vitamins E, And, corticosteroids. For elimination of cracks of a membrane of Bruch at a syndrome Grenblad — Strandberg use photocoagulation (see. Photocoagulation of an eye ), at early stages — an argonlazerkoagulyation (see. Laser ).
Forecast depends on extent of damage of eyes and internals. In rare instances the centers of defeat on skin are resolved spontaneously then there are sites of an atrophy.
Prevention it is directed to prevention of a progressirov of a stub of process and consists in carrying out repeated courses of vitamin therapy. Sick P. e. are subject to dispensary observation of the dermatologist, ophthalmologist and therapist.
Bibliography: Dermatological sindromologiya, under the editorship of R. S. Babayants, page 72, Yerevan, 1974; Zverkova F. A. About an elastic pseudoxanthoma at children, Vestn. dermas, and veins., No. 10, page 39, 1979, bibliogr.; Stepanova JI. And., N. I. and Yesenin K. P. Butterfish. An elastic pseudoxanthoma to Darya, in the same place, No. 9, with „48, 1980; SönnichsenN. u. Zabel R. Hautkrankheiten, Diagnostik und Therapie, S. 291, Lpz., 1976; Y a-r about t t i C. e. a. Lo pseudoxantoma elas-tico, G. ital. Derm. Vener., v. 116, p. 99, 1981, bibliogr.
V. I. Samtsov; V. V. Vladimirov (tsvetn, fig.).