Table 1.

Key features and differential diagnoses of CBDC

FEATUREDESCRIPTION
Clinical presentation
  • Average age of pediatric patients with CBDC is 4.5 y; neonatal cases have been described

  • Patient presents with tense clear or hemorrhagic blisters and vesicles 0.3 to 2.0 cm in diameter; new blisters may form near previous erosions (string-of-pearls or crown-of-jewels sign)

  • Cases with blisters solely in the genital area have been described

Differential diagnoses for blistering in children
  • Other autoimmune bullous disease: pemphigus vulgaris, dermatitis herpetiformis, bullous pemphigoid, epidermolysis bullosa acquisita, bullous lupus

  • Bacterial infections: bullous impetigo, staphylococcal scalded skin syndrome

  • Viral infections: herpes simplex virus, varicella zoster virus

  • Mechanical trauma: sucking blisters, friction blisters

  • Bites: arthropod, bullous scabies

  • Inflammatory disease: bullous mastocytosis, bullous lichen sclerosus, bullous lichen planus

  • Genetic disease: epidermolysis bullosa, porphyria, incontinentia pigmenti

HistologySubepidermal blister with neutrophils and occasional eosinophils in the papillary dermis
ImmunofluorescenceStriking linear deposition of IgA along the basement membrane; IgG, IgM, and C3 may also be present as weaker bands
  • C3—complement component 3, CBDC—chronic bullous disease of childhood, Ig—immunoglobulin.