[Herpetic-like worsening of an epidermolysis bullosa simplex during pregnancy].

BACKGROUND

Episodes of hereditary epidermolysis bullosa simplex are usually provoked by repeated cutaneous traumas or exposure to heat. We report a case of hereditary epidermolysis bullosa simplex worsened during pregnancy.

METHODS

A 21 year-old woman suffering from hereditary epidermolysis bullosa simplex presented with unusual symptoms in the form of an extensive vesicular-bullous eruption at two months of her first pregnancy. Standard histological examination revealed an eosinophilic infiltrate in the bullae. The global results of the initial examinations suggested a herpetic-like episode of epidermolysis bullosa as is observed during the Dowling-Meara form of the disease.

CONCLUSIONS

Various physiopathological hypotheses can be advanced to explain the worsening of the eruption during pregnancy: a mechanical origin with scratching of pruritus, enhanced fragility of the inter-keratinocyte bridges secondary to edema provoked by salt-water retention or a loss in antigenic tolerance to muted keratins 5 and 14, as in pemphigoid gestationis. The presence of eosinophils in the dermis and epidermis may also participate directly in the formation of bullae due to the release of cationic proteins.