Pseudoxanthoma Elasticum

PSEUDOXANTHOMA ELASTICUM (elastic pseudoxanthoma), a rare skin disease described in detail by Darier (1896). P. e. can develop at any age, affects both sexes equally, and sometimes has a familial character. P. e. is localized on covered parts of the body, mainly on the anterior and lateral sides of the neck, chest, in the navel area, lower abdomen, in the axillary regions, and elbow flexures. The rash is almost always symmetrical and consists of slightly elevated round, polygonal, and elliptical soft flat papules and spots, and in places, stripes forming a network; the color resembles xanthomatous, from ivory to orange-yellow and brownish. The affected skin is flaccid, thickened, in places, on the contrary, appears atrophic; there are no subjective symptoms. In addition to typical P. e., a number of cases of "atypical" P. e. have been described, the relationship of which to the latter is disputed by many. The histological picture is very characteristic: in the papillary and reticular layers of the skin, there are limited foci of degeneration and breakdown of elastic tissue, the fibers of which are torn, twisted, in places thickened and multiplied, their ability to stain is sharply changed, etc. (elastoclasis or elastorrhexis of Darier), xanthoma cells are not present. The etiology and pathogenesis are unclear. The essence of the process is apparently in the primary lesion of elastic tissue. Some (Arzt, Bettmann, Kaufmann-Wolf and others) advocate for the congenital nature of the disease, considering P. e. a hamartoma in the sense of Albrecht or a special type of nevus (naevus elasticus of Levandovsky-Gutman, elastoma of Juliusberg), Darier attributes P. e. to skin atrophies. There is also an opinion about the possible role of syphilis and endocrine disorders. Cases of the simultaneous existence of P. e. and a rare congenital eye disease-"Pigmentstreifenerkrankung des Augenhintergrundes" ("angioid streaks")-confirm the point of view of the first authors. Gronblad on this basis even speaks of a general disease of the elastic substance of the body. The course of P. e. is chronic, slowly progressive. For treatment, electrocoagulation can be recommended.