EPIDERMOLYSIS RULLOSA HEREDITARIA Clinical Picture.—The following is the typical pathologic picture as it was first described by Goldscheider in 1SS2 and later by Kiffiner: Suddenly, often in summer, there occur vesicles on the normal skin at all place.s of the body iron] a minimal external eause (friction, pressure, etc.) without inflammation. The fluid they contain is generally limpid, only rarely blood stained serum. The affection attacks particularly the feet, calves, hands, but may occur at any part of the body. The dis position to this cystic formation is congenital and bas been observed through several generations (through four and Eve by Valentin and Ronaiuti). The affection occurs chiefly in summer and especially when the skin has been exposed to some irritation. Should the feet be affected, the complaints may be so severe that the children are unable to walk. The affection strangely attacks almost exclusively children of German descent, a fact for which cient explanation has not as yet been forthcoming. The cysts heal without leaving any sear worth mentioning: a pigmentation only rarely remains. The affection may persist for life and cause considerable trouble. Aside from the typical form, a series of authors (llarier, Hallopeau, Fox, etc.) have described a second group, which materially differs from the first. This frequently
shows keloid cicatrices, miliary cysts, very pronounced pigmentation, trophic disturbances, frequent involvement of the mucous membranes, nail affections, etc. (llallopeau: Forme bulleuse dystrophique). Whether this second form really belongs here or not is still a matter of contention. Vidal and Hallopeau have even described a third form (la forme fruste), in which the cystic formations are of comparatively- less importance, or rather exist only in infancy, and in which instead the atrophic changes of the skin play the principal nile.
The etiology of the affection is unknown and heredity plays a most important part. Thus -Valentin observed 11 members of one family suffering from it in four generations, 13onaiuti saw 31 afflicted in a family of 63 members, Bettmann saw 10 cases among the offspring from one grandmother.
The. prognosis in spite of chronicity in isolated cases, is relatively favorable, whereas the rases which are accompanied with trophoneurotic disturbances are distinctly less favorable.
Treatment.—Treatment is comparatively helpless. In one case Jarisch observed a favorable effect from arsenic, otherwise the treat ment can only be directed toward the protection of the skin and the alleviation of the subjective complaints.