Lichen Planopilaris Pemphigoides: A Novel Bullous Dermatosis Due to Programmed Cell Death Protein-1 Inhibitor Therapy

Abstract

Lichen planus pemphigoides (LPPemph), apart from bullous pemphigoid, is a rare bullous dermatosis that can be induced by programmed cell death protein-1 (PD-1)/PD ligand 1 (PD-L1) inhibitors. The primary location of PD-1/PD-L1 inhibitor-induced LPPemph has previously only been reported at the nonfollicular dermal-epidermal junction. We present a case of nivolumab-induced LPPemph with an intense perifollicular lichenoid reaction, prominent multifocal perifollicular clefting, which in addition, was also accompanied by linear IgG and C3 immunofluorescence deposits along the dermal-epidermal junction as well as demonstrating a perifollicular pattern. Intriguingly, the serological study of BP180 and BP230 antibodies was negative, suggesting the presence of additional novel antibodies, which primarily favor hair follicles and may contribute to the pathogenesis. Therefore, we consider this entity a novel variant of PD-1/PD-L1 inhibitor-induced bullous dermatosis. To the best of our knowledge, this is the first report that highlights perifollicular bullae accompanied by immunofluorescence findings in a PD-1/PD-L1 inhibitor-induced lesion. We propose a new immunotherapy associated entity, lichen planopilaris pemphigoides, and emphasize the significance of perifollicular changes in the pathogenesis.

Pembrolizumab-Induced Lichen Planus Pemphigoides in a Patient with Metastatic Adrenocortical Cancer: A Case Report and Literature Review

“…In one of these cases, however, an immunoblotting revealed IgG antibodies specific to C-terminus of BP180 [ 21 ]. The second patient with negative BP180 and BP230 antibodies had a unique presentation of follicular immunobullous dermatosis, as opposed to the subepidermal bullae at the nonfollicular dermal–epidermal junction observed in the other reported LPP cases [ 18 ]. The authors proposed the role of a novel antibody mediating the presentation of LPP at the hair follicle—suggesting a unique target at the perifollicular basement membrane that triggered a lichenoid reaction [ 18 ].…”

Liquen Plano Penfigoide: una entidad clinicopatológica poco frecuente

“…En cuanto a la histopatología, los cuerpos de Civatte y los coloides son más prominentes en el LPA, el infiltrado inflamatorio tendrá un predominio de eosinófilos en el LPP y la inmunofluorescencia para el LPA será negativa (9) . Dentro del PA, otro diagnóstico que debe tenerse en cuenta, no habrá lesiones de LP y pese a que los antígenos del PA y del LPP son los mismos, los epítopos objetivos son diferentes para cada entidad (10) . Además del PA y el LPA, otras dermatosis forman parte del diagnóstico diferencial; por ejemplo, el lupus eritematoso cutáneo subagudo atípico puede presentarse con características clínicas similares al LPP, lo que subraya la importancia de un estudio inmunológico completo para demostrar la presencia de autoanticuerpos (2) .…”

Primary localized cutaneous lichen myxedematosus with light chain‐restricted plasma cells: A distinct variant of the localized form of lichen myxedematosus

“…The immunoglobulins produced by light chainrestricted plasma cells in our localized LM cases are comparable to the "unknown serum factor" and account for the pathogenesis. The overproduction of immunoglobulins has been observed in MZL (immunocytomas) as Russell and Dutcher bodies, in localized cutaneous amyloidosis, and in other autoimmune diseases, such as Graves disease, paraneoplastic pemphigus, and lichen planopilaris pemphigoides 26. It is critical to identify the presence of the light chain-restricted plasma cells in lesions of LM.…”