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.