title: Visual diagnosis: 4-month-old girl with diffuse, varied dermatitis and bullae.
abstract: 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.
authors.affiliation: Department of Pediatrics, Yale New Haven Hospital, New Haven, CT.
authors.forename: Sarah
authors.initials: S
authors.lastname: Fuchs
authors.affiliation: Department of Pediatrics, Yale New Haven Hospital, New Haven, CT.
authors.forename: Alissa
authors.initials: A
authors.lastname: Baker
authors.affiliation: Yale University School of Medicine, New Haven, CT.
authors.forename: Lucinda S
authors.initials: LS
authors.lastname: Liu
authors.affiliation: Pediatric Dermatology Fellow, Children's Mercy Hospital, Kansas City, MO.
authors.forename: Brea
authors.initials: B
authors.lastname: Prindaville
authors.affiliation: Yale University School of Medicine, New Haven, CT.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Antaya
mesh_terms: D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007223:Infant; D007958:Leukocyte Count; D010391:Pemphigoid, Bullous / Q000175:diagnosis*; D011537:Pruritus / Q000209:etiology; D012867:Skin / Q000276:immunology / Q000378:metabolism
chemical_list: D007074:Immunoglobulin G