Childhood epidermolysis bullosa acquisita: a novel variant with reactivity to all three structural domains of type VII collagen.

Full Abstract

Most patients with epidermolysis bullosa acquisita develop an autoimmune response to the non-collagenous (NC) 1 domain of type VII collagen. We report a 4-year-old girl of white European descent presenting with widespread blistering disease involving the face, hands, genital area and oral mucosa. Histopathology revealed subepidermal blisters, and linear deposits of IgG and C3 were seen along the dermal-epidermal junction on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. On indirect IF microscopy, circulating autoantibodies exclusively stained the dermal side of 1 mol L-1 NaCl-split skin. The patient's IgG autoantibodies labelled a 290-kDa protein on Western blotting of dermal extracts, and reacted with the NC1, NC2 and triple helical domains of type VII collagen on immunoblotting of recombinant and cell-derived fragments obtained by pepsin and collagenase digestion of the full-length protein. Oral methylprednisolone and dapsone led to clearance of lesions, which healed with mild scarring and milia formation. Treatment was discontinued after 1 year and the patient has now been in remission for more than 3 years.

Author information

Author/s: Schmidt, E (E); Höpfner, B (B); Chen, M (M); Kuhn, C (C); Weber, L (L); Bröcker, E-B (EB); Bruckner-Tuderman, L (L); Zillikens, D (D);

Affiliation: Department of Dermatology, University of Würzburg, Josef-Schneider-Str. 2, 97080 Würzburg, Germany.

Journal and publication information

Publication Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't

Journal: The British journal of dermatology (Br J Dermatol), published in England. (Language: eng)

Reference: 2002-Sep; vol 147 (issue 3) : pp 592-7

Dates: Created 2002/09/04; Completed 2002/11/22; Revised 2006/11/15;

PMID: 12207608, status: MEDLINE (last retrieved date: 2/18/2009)

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