Epidermolysis bullosa.

The term epidermolysis bullosa is applied to a group of conditions where vesicles occur most commonly on the skin but sometimes on mucous membranes. The content of these vesicles is serous but may become turbid or haemorrhagic, and secondary infection sometimes occurs. Apart from a type that may follow administration of sulphonamides (Bloom, I953), all forms appear to be genetically determined at single gene loci. Further, all cases appear to be determined by autosomal mutations, with the exception of the patients in one family where epidermolysis bullosa appears as part of a widespread syndrome determined by a sex-linked gene. This family first described by Mendes da Costa and Van der Valk (I908) has subsequently been reported by various authors over a number of years under varying titles: perhaps the most generally used at the present time being dystrophia bullosa, typus maculatus (Woerdeman, I958).