Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering

Abstract

Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17). However, accurately establishing the diagnosis is dependent on a careful correlation between the clinical, histological and immunological features of the disease. Therefore, we present an up to date summary of the epidemiology and etiopathogenesis of LPP, before illustrating the predisposing and precipitating factors implicated in the development of the disease. In addition to a selective literature search, we compare reports of potential drug-induced cases of LPP with pharmacovigilance data available via OpenVigil. We subsequently outline the cardinal clinical features, important differential diagnoses and current treatment options. We conclude by demonstrating that an improved understanding of LPP may not only lead to the development of novel treatment strategies for the disease itself, but may also shed new light on the pathophysiology of more common and treatment-refractory autoimmune blistering diseases.

Case of lichen planus pemphigoides after pembrolizumab therapy for advanced urothelial carcinoma

“…In our case, a good therapeutic response of pembrolizumab was not observed, unlike previous cases of LPP induced by anti‐PD‐1 monoclonal antibodies 3,5 . The situation of T‐cell responses in LPP still remains unclear 1 . Pembrolizumab may be an important cofactor, but not the direct trigger of LPP.…”

“…Lichen planus pemphigoides (LPP) is an autoimmune subepidermal blistering disease associated with lichenoid skin changes, and various predisposing and precipitating factors have been reported 1 . Here, we report a rare case of LPP after pembrolizumab therapy for advanced urothelial carcinoma.…”

“…The diagnosis of LPP is made by the combination of the typical clinical features and the demonstration of autoantibodies binding to the dermal–epidermal junction 1 . The development of LPP has been reported to be associated with medication and/or pre‐existing medical conditions 1 .…”

“…The diagnosis of LPP is made by the combination of the typical clinical features and the demonstration of autoantibodies binding to the dermal–epidermal junction 1 . The development of LPP has been reported to be associated with medication and/or pre‐existing medical conditions 1 . Pembrolizumab, an anti‐programmed death 1 (PD‐1) monoclonal antibody, has been demonstrated to be effective and safe for the treatment of advanced urothelial carcinoma 2 .…”

Lichen planus pemphigoides after body tattooing

“…The subsequent development of lichen planus pemphigoides might be due to exposure of antigens BPAg1 (BP230) and BPAg2 (BP180 or type XVII collagen) following basal cell and basement membrane damage in lichen planus; generation of autoantibodies against these antigens then leads to bullae formation 16 . Lichen planus pemphigoides is a rare condition with an estimated prevalence of 1:1000 000 17 . Lichen planus pemphigoides is characterized by the development of bullae after lichenoid skin changes 17 and differs from bullous pemphigoid in that lichen planus pemphigoides has a younger average age of onset 18 .…”