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Table 1 Emergencies in EB

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

EmergencySymptomsEB subtypeKey references
SepsisFever, increased CRP, leukocytosis, positive blood culturesAny severe EB subtype[1,2,3]
Acute feeding inability in newborns/infantsFeeding refusal, sialorrhea, blisters in the oral cavityDEB[4, 5]
Acute airway obstructionShortness of breath, stridor, distress, dusky skin colorationJEB[6,7,8,9]
Acute esophageal obstructionPainful dysphagia, aphagia, sialorrhea, regurgitationRDEB[4, 10,11,12]
Acute urinary retentionInability to pass urine, lower abdominal distension and painJEB, DEB, KEB[13,14,15,16]
Corneal erosionPain, photophobia, blepharospasm, tearingJEB, DEB, KEB[5, 17,18,19,20,21]
  1. CRP C-reactive protein, DEB dystrophic epidermolysis bullosa, RDEB recessive DEB, JEB junctional epidermolysis bullosa, KEB Kindler epidermolysis bullosa
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