An 8 year old boy developed progressive blistering of the face, scalp and neck. In many areas blisters were clustered in rings around central crusts typical of the "string of pearls" seen in linear IgA dermatosis. He was well and without symptoms except for minimal itching. A skin biopsy from the edge of a blister on the neck showed a subepidermal blister, and direct immunofluorescence on a salt split specimen demonstrated an intense linear band of IgG on the dermal side of the basement membrane zone. Indirect immunofluorescence showed intense staining on the dermal side of the basement zone as well consistent with epidermolysis bullosa acquisita.
tense blisters on a red base clustered around a central crust