J Korean Assoc Pediatr Surg.  1997 Jun;3(1):77-82. 10.13029/jkaps.1997.3.1.77.

Congenital Pyloric Atresia with Junctional Epidermolysis Bullosa: a case report

• Cho MH
• Han SJ

• KMID: 2422272
• DOI: http://doi.org/10.13029/jkaps.1997.3.1.77

Abstract

The association of pyloric atresia and epidermolysis bullosa(EB) in newborn is rare and inheritant as an autosomal recessive trait. We report a newborn girl with pyloric atresia and epidermolysis bullosa. Blisters were noted on her skin at birth, especially in pressure-exposed area, and later on the oral mucosa. Junctional epidermolysis bullosa was confirmed by light microscopy and electron microscopy. Radiography revealed pyloric atresia. Segmental resection of 1.5 cm and gastroduodenostomy were carried out at 4 days of age. Protein loosing enteropathy developed after oral feeding. The frequency of episodes of nonscarred blisters and the severity and duration improved significantly with time. The protein loosing enteropathy was persistent, and at 1 year of age, her growth is markedly retarded.

Keyword

Pyloric atresia; Epidermolysis bullosa

MeSH Terms

Blister
Epidermolysis Bullosa
Epidermolysis Bullosa, Junctional*
Female
Humans
Infant, Newborn
Microscopy
Microscopy, Electron
Mouth Mucosa
Parturition
Radiography
Skin