Challenges and pitfalls between lichen planus pemphigoides and bullous lichen planus

Abstract

Lichen planus pemphigoides (LPP) and bullous lichen planus (BLP) are rare dermatoses, which are characterised by blisters and lichenoid lesions. Their clinical presentation is heterogenous, displaying overlapping features or mimicking other dermatological diseases. Therefore, diagnosis can often be challenging, requiring a thorough dermatological examination along with distinctive histological and immunopathological characteristics. Lichenoid degeneration of the basal epidermis exposes various antigens of the dermal-epidermal junction in LPP, resulting in the breakdown of immune tolerance, hence, the production of autoantibodies against type XVII collagen. Conversely, no pathogenic autoantibodies are detected in BLP. However, some cases of mucosal lichen planus might display immunopathological features suggestive of autoimmune blistering diseases. Therefore, a better understanding of the pathophysiology of these two distinct dermatoses is imperative. The aim of this review was to provide a summary of the current knowledge on the clinical hallmarks, diagnosis and available therapeutic options in LPP and BLP.

Effective treatment of refractory lichen planus pemphigoides with a Janus kinase-1/2 inhibitor

“…Dear Editor, Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disease clinically and histopathologically characterized by features of LP and bullous pemphigoid (BP). 1,2 We describe a case of refractory LPP successfully treated with the selective and reversible Janus kinase (JAK)-1/2 inhibitor, baricitinib.…”

“…LPPis an autoimmune bullous dermatosis with an estimated prevalence of 1 in 1 000 000. 1,2 Once regarded as a variant of BP and/or LP, it is now considered a distinct clinical entity. 1 LPP is characterized by autoantibodies against Type XVII collagen (COL17), with its pathognomonic feature being an association with LP, supported by the demonstration of IgG and complement component C3 deposition at the dermoepidermal junction.…”

“…1,2 Once regarded as a variant of BP and/or LP, it is now considered a distinct clinical entity. 1 LPP is characterized by autoantibodies against Type XVII collagen (COL17), with its pathognomonic feature being an association with LP, supported by the demonstration of IgG and complement component C3 deposition at the dermoepidermal junction. 2 Additional autoantibodies directed against desmoglein 1, BP230 and two unidentified antigens with molecular weights of 130 and 200 kDa have been implicated in LPP pathogenesis.…”

“…2 Additional autoantibodies directed against desmoglein 1, BP230 and two unidentified antigens with molecular weights of 130 and 200 kDa have been implicated in LPP pathogenesis. 1,2 Treatment of LPP remains challenging. Several agents, including topical/oral corticosteroids, dapsone, acitretin, azathioprine, ciclosporin and mycophenolate mofetil have been used, with varying degrees of success.…”

“…Several agents, including topical/oral corticosteroids, dapsone, acitretin, azathioprine, ciclosporin and mycophenolate mofetil have been used, with varying degrees of success. 1,2 In patients with recalcitrant disease, treatment options are limited.…”