The clinical evolution of lichen planus pemphigoides

Abstract

Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disease characterized by lichenoid and bullous lesions. LPP is generally thought to be idiopathic, possibly related to medication or malignancy, or potentially the result of long-standing lichenoid inflammation damaging the basement membrane zone leading to epitope spreading. The histological appearance of lichenoid and bullous lesions in LPP resembles findings of lichen planus and bullous pemphigoid, respectively. We present a case of LPP in a 64-year-old woman with a brief historical review of the establishment of LPP as a separate disease entity and a discussion of similarities and differences of LPP with other lichenoid and blistering dermatoses.

Keywords: Blister; bullous; lichen; pemphigoides; planus.

Figure 1.

(a) The patient’s back upon initial presentation to the primary care physician showing numerous black macules, patches, papules, and plaques. (b) The patient’s back upon initial presentation to dermatology highlighting the progression of lesions to darker violaceous and black colors with noticeable erosions. (c) Close up showing a tense bulla. (d) Punch biopsy of lichenoid lesion demonstrating orthohyperkeratosis overlying an irregularly acanthotic epidermis with a thickened granular cell layer with focal parakeratosis overlying the lesion. A lymphoplasmacytic inflammatory infiltrate within the dermis along with melanophages surrounding the superficial vascular plexus can be seen. Numerous eosinophils are present within the superficial dermis and several within the epidermis. (e) Punch biopsy of bullous lesion demonstrating subepidermal bullae with eosinophils and lymphocytes in the dermal inflammatory infiltrate.