Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Table 1 Emergencies in EB

Emergency	Symptoms	EB subtype	Key references
Sepsis	Fever, increased CRP, leukocytosis, positive blood cultures	Any severe EB subtype	[1,2,3]
Acute feeding inability in newborns/infants	Feeding refusal, sialorrhea, blisters in the oral cavity	DEB	[4, 5]
Acute airway obstruction	Shortness of breath, stridor, distress, dusky skin coloration	JEB	[6,7,8,9]
Acute esophageal obstruction	Painful dysphagia, aphagia, sialorrhea, regurgitation	RDEB	[4, 10,11,12]
Acute urinary retention	Inability to pass urine, lower abdominal distension and pain	JEB, DEB, KEB	[13,14,15,16]
Corneal erosion	Pain, photophobia, blepharospasm, tearing	JEB, DEB, KEB	[5, 17,18,19,20,21]

CRP C-reactive protein, DEB dystrophic epidermolysis bullosa, RDEB recessive DEB, JEB junctional epidermolysis bullosa, KEB Kindler epidermolysis bullosa

ISSN: 1750-1172