Conflict of interest: none declared.
Epidermolysis bullosa pruriginosa in association with lichen planopilaris
Article first published online: 13 NOV 2009
© 2009 The Author(s). Journal compilation © 2009 British Association of Dermatologists
Clinical and Experimental Dermatology
Volume 34, Issue 8, pages e825–e828, December 2009
How to Cite
Almaani, N., Liu, L., Perez, A., Robson, A., Mellerio, J. E. and McGrath, J. A. (2009), Epidermolysis bullosa pruriginosa in association with lichen planopilaris. Clinical and Experimental Dermatology, 34: e825–e828. doi: 10.1111/j.1365-2230.2009.03568.x
- Issue published online: 13 NOV 2009
- Article first published online: 13 NOV 2009
- Accepted for publication 30 March 2009
Epidermolysis bullosa pruriginosa (EBP) is a clinical variant of dominant or occasionally recessive, dystrophic epidermolysis bullosa (EB). Clinically, intense pruritus on a background of inherited skin fragility often leads to skin signs that resemble acquired inflammatory disorders such as hypertrophic lichen planus (LP) or nodular prurigo. Moreover, symptoms and signs may not appear until adult life, further compounding difficulties in distinguishing between inherited or acquired skin pathology. We describe a 61-year-old white British woman who developed EBP during her 40s, with lichenified plaques on the legs that resembled hypertrophic LP. Molecular screening of the COL7A1 gene showed a novel heterozygous glycine substitution in type VII collagen, designated p.G2290A, in keeping with dominant dystrophic EB. During her 50s, however, the patient developed new abnormalities with patchy scarring alopecia and perifollicular inflammation. Histological examination of a skin biopsy found features of lichen planopilaris. To our knowledge, this is the first example of a patient with EBP in whom the genetic disease does not merely resemble LP but is actually associated with coexisting acquired lichenoid skin pathology. Intriguingly, treatment with topical tacrolimus 0.03% led to marked improvement in the inflammation on the legs but had little effect on the scalp.