PEDIATRICS Vol. 78 No. 1 July 1986, pp. 172-174
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Junctional Epidermolysis Bullosa of the Larynx

MARGARET A. KENNA MD1, SYLVAN E. STOOL MD1, and SUSAN B. MALLORY MD2

1 Department of Otolaryngology, University of Pittsburgh School of Medicine and Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh
2 Departments of Dermatology and Pediatrics, University of Arkansas Medical Center and Arkansas Children's Hospital, Little Rock

Epidermolysis bullosa is a rare genetically determined, dermatologic disease in which minor trauma causes blister formation.1 A new variant of hereditary epidermolysis bullosa, generalized atrophic benign epidermolysis bullosa, junctional form, has been recently reported.2 Airway involvement has not been a notable feature of this disease. We report the first case of an infant having benign junctional epidermolysis bullosa with laryngeal involvement.

CASE REPORT

An 11-month-old white boy with known junctional epidermolysis bullosa and mild stridor since birth was referred by his dermatologist for increasing stridor of 24 hours duration. He was initially thought to have croup; however, conservative treatment with mist and racemic epinephrine did not improve his symptoms.




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S. R. Schaffer
Head and Neck Manifestations of Epidermolysis Bullosa
Clinical Pediatrics, February 1, 1992; 31(2): 81 - 88.
[Abstract] [PDF]