Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid.

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.