Open AccessChildhood epidermolysis bullosa acquisita: Confirmation of diagnosis by skin deficient in Type VII Collagen, enzyme-linked immunosorbent assay, and immunoblotting
Author(s) -
Nupur Goyal,
Raghavendra Rao,
C Balachandran,
Sathish Pai,
B.S. Bhogal,
Enno Schmidt,
Detlef Zillikens
Publication year - 2016
Publication title -
indian journal of dermatology/indian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.395
H-Index - 36
eISSN - 1998-3611
pISSN - 0019-5154
DOI - 10.4103/0019-5154.182420
Subject(s) - epidermolysis bullosa acquisita , medicine , autoantibody , dapsone , pathology , anchoring fibrils , staining , epidermolysis bullosa , dermatology , basement membrane , type iv collagen , cicatricial pemphigoid , immunofluorescence , antibody , pemphigoid , immunology , laminin , bullous pemphigoid , chemistry , biochemistry , cell
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.