Human Orf complicated by Epidermolysis Bullosa Acquisita

Summary

Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies. We describe the case of a patient who presented an ulceration on his finger ten days after a direct contact with a lamb during Eid al-Adha. Four weeks later, he developed a severe tense blistering eruption associated with mucous membrane erosions. Indirect immunofluorescence analysis using the patient's serum revealed circulating anti-basement membrane IgG that bound the dermal side of salt-split skin. Enzyme-linked immunosorbent assay (ELISA) was positive for recombinant immunodominant NC1 domain of type VII collagen. We finally diagnosed Epidermolysis Bullosa Acquisita complicating probable human Orf infection.

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