IgG Autoantibodies from a Lichen planus pemphigoides Patient Recognize the NC16A Domain of the Bullous Pemphigoid Antigen 180

Abstract

Lichen planus pemphigoides (LPP) most likely encompasses a heterogeneous group of subepidermal autoimmune blistering disorders occurring in association with lichen planus. We describe the case of a 49-year-old patient with features characteristic of LPP. Direct immunofluorescence microscopy studies demonstrated linear deposits of C3 along the cutaneous basement membrane, while circulating IgG autoantibodies directed against the epidermal side of skin separated by 1 M NaCl were detected. The patient’s serum contained IgG autoantibodies immunoblotting a recombinant form of bullous pemphigoid antigen 180 (BP180), but not the COOH-terminus of BP230. By using deletion mutants, it was found that IgG reactivity was restricted to the NC16A domain of BP180, the region harboring the major antigenic sites targeted by IgG autoantibodies from patients with the bullous pemphigoid group of disorders. Our findings provide support to the idea that a subset of patients with LPP have a distinct form of bullous pemphigoid associated with lichen planus.

Heterogeneous disease: A child case of lichen planus pemphigoides triggered by varicella

“…There is a controversy as to whether LPP is a heterogeneous disease or whether it represents the coexistence of lichen planus and bullous pemphigoid 3 . It most likely denotes the occurrence of lichen planus associated with distinct subepidermal autoimmune bullous disorders, including most frequently bullous pemphigoid 2 . Most of the immunoblotting studies on LPP reveal the major 230‐kDa bullous pemphigoid antigen and the minor 180‐kDa bullous pemphigoid antigen suggesting the coexistence of bullous pemphigoid 4 .…”

“…In contrast, very few studies demonstrate that the target antigen in LPP is not unique and has different properties, suggesting that LPP may be a heterogeneous disease 5–7 . Furthermore, the immunoreactants have usually been localized to the lamina lucida and the hemidesmosomal region, and less frequently, to the lamina densa and sub‐lamina densa region of the epidermal BMZ 2 . Most patients with LPP have circulating IgG anti‐BMZ antibodies, which deposit on the epidermal sides of salt‐split skin.…”

“…Light microscopy studies of papular lesions demonstrate features typical of lichen planus, while biopsies obtained from bullous lesions reveal a subepidermal blister. Direct immunofluorescence microscopy studies demonstrate linear deposits of C3 and/or immunoglobulin (Ig)G along the epidermal basement membrane zone (BMZ) 1,2 . Some authors consider LPP as a heterogeneous condition whereas most authors regard it as the combination of two diseases: lichen planus and bullous pemphigoid 2 .…”

Oral, Esophageal and Cutaneous Lichen Ruber Planus Controlled with Alitretinoin: Case Report and Review of the Literature

“…Lichen planus pemphigoides refers to a cross-over syndrome of LP and bullous pemphigoid [5] and associations with drug intake have been described (PUVA [39], UVB, paracetamol or ibuprofen [40], simvastatin [41] and ramipril [42,43]). Several reports show antibodies against 180, 130 and 200 kDa epidermal antigens [40,44,45,46,47,48,49,50,51]. Some reports also describe an overlap between LP and lupus erythematosus [52,53,54,55,56,57].…”

Localized Childhood Vulval Pemphigoid Treated with Tacrolimus Ointment

“…Direct immunofluorescence microscopy showed linear continuous deposits of IgG and C3 along the dermoepidermal junction (not shown). Search for circulating autoantibodies by indirect immunofluorescence using 1  M NaCl-separated normal human skin and immunoblotting of keratinocyte extracts remained negative [7, 8]. Based on the clinical and immunopathological findings, a diagnosis of cicatricial vulval pemphigoid was made, although definite classification within either the pemphigoid or the epidermolysis bullosa acquisita group was not possible [7].…”