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March 1954

EPIDERMOLYSIS BULLOSA HEREDITARIA LETALIS IN NEWBORN TWINS: Report of Two Cases with Failure to Respond Favorably to Cortisone

AMA Am J Dis Child. 1954;87(3):321-327. doi:10.1001/archpedi.1954.02050090309007

EPIDERMOLYSIS bullosa is a chronic hereditary condition of the skin and/or mucous membranes, characterized by the development at any time of life, usually early, of vesicles or bullae under the influence of trauma. This disease occurs in varying grades of severity, from the fatal to the mildly symptomatic.

Cockayne1 classified the condition into two main types, epidermolysis bullosa simplex and epidermolysis bullosa dystrophica. He subclassified the latter into the dominant form, the recessive form, and the types originally described by Guy and Wende, the "typhus maculatus" type described by Mendes da Costa and van der Valk, and the type described by Heinrichsbauer.5

Herlitz2 first named and collated cases of a distinct fatal type which he called "epidermolysis bullosa hereditaria letalis," including 8 of his own cases and 14 others, those of Mautner,3 Jenny,4 Heinrichsbauer,5 and Kuse.6

Herlitz stated that epidermolysis bullosa hereditaria letalis