BACKGROUND: Childhood localized vulvar pemphigoid has been recently reported in four girls. A fifth child with this proposed rare variant of bullous pemphigoid is described. Moreover, findings in the various immunopathologic studies we performed establish this entity as a true morphologic variant of bullous pemphigoid. OBSERVATIONS: In situ deposits of IgG in this patient's epidermal basement membrane zone localized to the epidermal side of 1 mol/L of saline-split skin. Moreover, the patient had circulating IgG autoantibodies that bound the epidermal side of 1 mol/L of saline-split skin in indirect immunofluorescence microscopy and immunoprecipitated the 230-kd bullous pemphigoid antigen from biosynthetically radiolabeled human keratinocyte extracts. These laboratory findings are identical to those documented in patients with the generalized "classic" form of bullous pemphigoid. CONCLUSIONS: This study demonstrates that a child with clinical, histopathologic, and immunopathologic features of localized vulvar pemphigoid had circulating autoantibodies that identify a specific keratinocyte antigen, the bullous pemphigoid antigen, which may serve as a molecular marker for this disease.
BACKGROUND: Childhood localized vulvar pemphigoid has been recently reported in four girls. A fifth child with this proposed rare variant of bullous pemphigoid is described. Moreover, findings in the various immunopathologic studies we performed establish this entity as a true morphologic variant of bullous pemphigoid. OBSERVATIONS: In situ deposits of IgG in this patient's epidermal basement membrane zone localized to the epidermal side of 1 mol/L of saline-split skin. Moreover, the patient had circulating IgG autoantibodies that bound the epidermal side of 1 mol/L of saline-split skin in indirect immunofluorescence microscopy and immunoprecipitated the 230-kd bullous pemphigoid antigen from biosynthetically radiolabeled human keratinocyte extracts. These laboratory findings are identical to those documented in patients with the generalized "classic" form of bullous pemphigoid. CONCLUSIONS: This study demonstrates that a child with clinical, histopathologic, and immunopathologic features of localized vulvar pemphigoid had circulating autoantibodies that identify a specific keratinocyte antigen, the bullous pemphigoid antigen, which may serve as a molecular marker for this disease.
Authors: H Rashid; A Lamberts; L Borradori; S Alberti-Violetti; R J Barry; M Caproni; B Carey; M Carrozzo; F Caux; G Cianchini; A Corrà; G F H Diercks; F G Dikkers; G Di Zenzo; C Feliciani; G Geerling; G Genovese; M Hertl; P Joly; A V Marzano; J M Meijer; V Mercadante; D F Murrell; M Ormond; H H Pas; A Patsatsi; C Prost; S Rauz; B D van Rhijn; M Roth; E Schmidt; J Setterfield; G Zambruno; D Zillikens; B Horváth Journal: J Eur Acad Dermatol Venereol Date: 2021-07-10 Impact factor: 6.166