Newborn with severe epidermolysis bullosa: to treat or not to treat?
Epidermolysis bullosa (EB) is an inherited skin disease with four main subtypes that cannot be distinguished clinically at birth. All subtypes may present with widespread life-threatening blisters and fragile skin, making treatment and handling of the newborn with EB challenging. The prognosis of EB depends on the subtype, and therefore maximum treatment is necessary until the final diagnosis is known. In this case, it took 2 weeks before a final diagnosis was reached. In the meantime, we had several ethical discussions on the treatment level. The most important issues were management of pain and nutrition. For immediate pain relief, intranasal fentanyl worked best and gabapentin was successfully used for chronic pain. The feeding difficulties were handled first by a nasogastric feeding tube. Later a normal feeding bottle proved to be adequate.
Department of Paediatric, Hospital of Southern Denmark, Aabenraa, Aabenraa, Denmark.
Martin Lehmann
ML
Boesen
Department of Dermatology and Allergy Center, University Hospital of Odense, Odense, Denmark.
Anette
A
Bygum
Department of Clinical Genetics, University Hospital of Odense, Odense, Denmark.
Jens Michael
JM
Hertz
Neonatal Intensive Care Unit, HC Andersen Child Hospital, Odense, Denmark.
Gitte
G
Zachariassen
D000588:Amines / Q000627:therapeutic use; D001768:Blister / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D059350:Chronic Pain / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D003509:Cyclohexanecarboxylic Acids / Q000627:therapeutic use; D004820:Epidermolysis Bullosa / Q000145:classification / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D016109:Epidermolysis Bullosa, Junctional / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D005283:Fentanyl / Q000627:therapeutic use; D000077206:Gabapentin; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018529:Nutritional Support; D059408:Pain Management; D012720:Severity of Illness Index; D012867:Skin / Q000473:pathology*; D005680:gamma-Aminobutyric Acid / Q000627:therapeutic use
D000588:Amines; D003509:Cyclohexanecarboxylic Acids; D005680:gamma-Aminobutyric Acid; D000077206:Gabapentin; D005283:Fentanyl