Reference Age/Sex Involvement Associated disorders or possible trigger factors Histology Immunofluorescence Treatment Skin on presentation Oral mucosa DIF* 180kDa** Rita E. Fisler, 2003 [9] 5m/M Palms, soles None DT# vaccine eosinophils IgG,C3, weaker IgA NA Topical steroids 8m/M Palms, soles Present None Mixed neutrophils, eosinophils IgG,C3 NA Prednisolone(2mg/kg/day), dapsone, mycophenolate mofetil 7 y/M Vulval area vagina None Cell-free IgG,C3 NA Topical steroids Stéphane Kuenzli, 2004 [16] 9m/F Wide spread, palms, soles vagina None eosinophils IgG,C3 present Prednisolone(1mg/kg/day), sulfapyridine Ting XIAO, 2007 [3] 3.5m/M face, trunk, hands and feet None DTP# vaccine neutrophils C3 present high-dose i.v. immunoglobulin Gino Tripodi, 2007 [17] 42m/F Not described present IBD Not described Not described NA Plasma exchange Maria Isabel Martinez-De Pablo, 2007 [18] 5m/M trunk, face, lower limbs present None eosinophils IgG,C3,IgA(faint) NA Prednisolone(2mg/kg/day) 5m/M hands, feet, and trunk None None eosinophils IgG,C3,IgA(faint) NA Prednisolone(1mg/kg/day) 12m/M scalp, trunk, limbs, palms, soles None None Not described IgG,C3 NA Dapsone(1mg/kg/day), Prednisolone(1mg/kg/day) 4m/M abdomen, limbs, scalp, palms, soles None None eosinophils IgG,C3 NA Prednisolone(1mg/kg/day) Ana María Sáenz, 2007[19] 15m/M trunk, neck, limbs, face, palm, soles Present None lymphocytes,eosinophils IgG,C3 present Prednisolone(1mg/kg/day) V. Majmudar, 2008 [20] 10w/F scalp, trunk, inner thighs None DTaP#/IPV#/Hib# vaccine eosinophils IgG,C3 present Prednisolone(2mg/kg/day) Zulal Erbagci, 2008 [6] 6m/M palms, soles, lower extremities, scalp and ear lobes present Hyper-IgE syndrome eosinophils IgG,C3 NA Prednisolone(1mg/kg/day), oral antibiotics Moben Mirza, 2008 [21] 7y/M glans penis None None lymphocytes, plasma cells and rare eosinophils IgG,C3 NA corticosteroids Karen A. Marcus, 2009 [22] 3y/F Trunk, extremities None VZV# vaccine eosinophils C3,weaker IgG, IgM present Prednisolone(2mg/kg/day), Dapsone(1mg/kg/day) Tomoko TOYAMA, 2009 [23] 5m/F trunk, extremities, hands and feet None DTP# vaccine lymphocytes,eosinophil IgG,IgM,C3 present Topical steroids 5m/F face, fingers, trunk, extremities, palm, soles None BCG vaccine Lymphocytes, eosinophils IgG present Topical steroids Fox JC, 2010 [8] 16y/F >70% of her total body surface area None atopy eosinophils NA NA Prednisolone(1mg/kg/day), mycophenolate mofetil (MM), topical agent The present case, 2012 21m/M face, trunk, limbs, groin area, palm, soles None None Neutrophils IgG,C3 NA Prednisolone (0.5 mg/kg/day) NA: non available *DIF: direct immunofluorescence **180kDa: BP 180, type XVII collagen, components of junctional adhesion complexes called hemidesmosomes # DT: diphtheria and tetanus vaccine; DTP: diphtheria, tetanus and pertussis vaccine; DTaP: Diphtheria and tetanus toxoid with acellular pertussis vaccine; IPV: inactivated polio vaccine; Hib: haemophilus influenzae type b vaccine; VZV: varicella vaccine

Table 1: Summary of Childhood bullous pemphigoid case reports since 2003.