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
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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
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Histology | Subepidermal blister with neutrophils and occasional eosinophils in the papillary dermis |
Immunofluorescence | Striking linear deposition of IgA along the basement membrane; IgG, IgM, and C3 may also be present as weaker bands |