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July 2002

Pyloric Stenosis and Pyloric Atresia: The Same Pathogenesis?

Author Affiliations

Not Available

Arch Dermatol. 2002;138(7):979. doi:10.1001/archderm.138.7.979

I read with interest the article by Dereure et al1 that appeared in the "Vignettes" section of the May 2001 issue of the ARCHIVES in which they report a case of dystrophic epidermolysis bullosa associated with congenital absence of skin and pyloric stenosis. I partly disagree with the comment on this case. Pyloric stenosis is actually not a minor form of atresia. Infantile pyloric stenosis, or congenital hypertrophic pyloric stenosis, is a relatively common disorder with a high familial incidence as well as a marked male predominance. Its pathogenesis remains unclear. This condition requires abdominal surgery in the first few months of life. In contrast, pyloric atresia is rare. Atresia is classified according to 3 types.2 The congenital mucosal membrane type, which is the most common variant, consists of webs and diaphragms, but in epidermolysis bullosa associated with pyloric atresia, the longitudinal segmental variant of atresia predominates.2 Pathological examination of pyloric stenosis and atresia indicates that the pathogenesis of these 2 conditions is mediated by distinct pathways. The sporadic association between pyloric stenosis and dystrophic epidermolysis bullosa may be coincidental. The pyloric abnormality should be better analyzed to argue the case for the absence of strict correlation between the congenital gastric outlet obstruction and a specific epidermolysis bullous variant. The case reported by Dereure and colleagues is clearly different from the epidermolysis bullosa–pyloric atresia syndrome.