Childhood Epidermolysis Bullosa Acquisita: Confirmation of Diagnosis by Skin Deficient in Type VII Collagen, Enzyme-linked Immunosorbent Assay, and Immunoblotting.
Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Direct immunofluorescence (IF) microscopy showed linear deposition of IgG and C3 along the basement membrane zone (BMZ); indirect IF microscopy on salt-split skin revealed staining of IgG to the dermal side of the split. The patient's serum did not show BMZ staining in recessive dystrophic epidermolysis bullosa skin deficient for Type VII collagen, thus confirming autoantibody reactivity against Type VII collagen. Circulating antibodies against the immunodominant noncollagenous 1 domain of Type VII collagen were detected by ELISA and immunoblotting studies. The patient was treated with oral corticosteroids and dapsone with good improvement.
Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
Nupur
N
Goyal
Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
Raghavendra
R
Rao
Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
C
C
Balachandran
Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
Sathish
S
Pai
Department of Immunodermatology, St. John's Institute of Dermatology, St. Thomas Hospital, London.
Balbir S
BS
Bhogal
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Enno
E
Schmidt
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Detlef
D
Zillikens
Childhood epidermolysis bullosa acquisita; Type VII collagen; noncollagenous domain 1