Childhood epidermolysis bullosa acquisita

Confirmation of diagnosis by skin deficient in Type VII Collagen, enzyme-linked immunosorbent assay, and immunoblotting

Nupur Goyal, Raghavendra Rao, C. Balachandran, Sathish Pai, Balbir S. Bhogal, Enno Schmidt, Detlef Zillikens

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)329-332
Number of pages4
JournalIndian Journal of Dermatology
Volume61
Issue number3
DOIs
Publication statusPublished - 01-05-2016

Fingerprint

Epidermolysis Bullosa Acquisita
Collagen Type VII
Immunoblotting
Enzyme-Linked Immunosorbent Assay
Skin
Fluorescence Microscopy
Basement Membrane
Autoantibodies
Immunoglobulin G
Epidermolysis Bullosa Dystrophica
Staining and Labeling
Dapsone
Direct Fluorescent Antibody Technique
Blister
Indirect Fluorescent Antibody Technique
Foot
Leg
Adrenal Cortex Hormones
Hand
Salts

All Science Journal Classification (ASJC) codes

  • Dermatology

Cite this

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abstract = "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.",
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Childhood epidermolysis bullosa acquisita : Confirmation of diagnosis by skin deficient in Type VII Collagen, enzyme-linked immunosorbent assay, and immunoblotting. / Goyal, Nupur; Rao, Raghavendra; Balachandran, C.; Pai, Sathish; Bhogal, Balbir S.; Schmidt, Enno; Zillikens, Detlef.

In: Indian Journal of Dermatology, Vol. 61, No. 3, 01.05.2016, p. 329-332.

Research output: Contribution to journalArticle

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