• Pediatric autoimmune blistering disorders are exceedingly rare. Of these, childhood bullous pemphigoid (CBP) is the most common IgG-mediated subepidermal bullous disease in the pediatric population. Tense acral blisters, especially on the soles and palms, are characteristic of the infantile presentation. • Patients with CBP present with varied dermatoses, making clinical diagnosis alone difficult. • Definitive diagnosis is made with direct immunofluorescence revealing linear deposition of IgG and/or C3 at the basement membrane zone (BMZ) or indirect immunofluorescence revealing IgG antibodies reacting with the BMZ. • First-line treatment is oral prednisolone dosed at 1 to 2 mg/kg and then tapered slowly to avoid rebound disease. The length of treatment depends on the rate of remission.
|Original language||English (US)|
|Journal||Pediatrics in review|
|State||Published - Aug 2014|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health