Lichen Planus Pemphigoides Research Articles

A novel epitope within the BP180 NC16A domain is targeted by autoantibodies in lichen planus pemphigoides

A novel epitope within the BP180 NC16A domain is targeted by autoantibodies in lichen planus pemphigoides

Lichen planus pemphigoides: another paraneoplastic bullous disease?

Abstract Background Despite a long-standing controversy regarding the classification of lichen planus associated with blistering, it seems likely today that bullous lichen planus and lichen planus pemphigoides are separate entities. Patients In this presentation two patients are described: a 56-year-old female with bullous lichen planus developed on persisting lesions over a period of 1 year and an 83-year-old female who developed lichen planus pemphigoides during the course of thyroid-gland carcinoma. Conclusion Each of these two entities has its own clinical, histological, immunohistological, and immunobiochemical features. Also, the outcomes seem to be different since lesions of bullous lichen planus usually resolve after a rather short period of time; persistent lesions may possibly develop into squamous cell carcinoma. In contrast, lichen planus pemphigoides is likely to be a paraneoplastic disorder.

Lichen planus pemphigoides: another paraneoplastic bullous disease?

AbstractBackground Despite a long‐standing controversy regarding the classification of lichen planus associated with blistering, it seems likely today that bullous lichen planus and lichen planus pemphigoides are separate entities.Patients In this presentation two patients are described: a 56‐year‐old female with bullous lichen planus developed on persisting lesions over a period of I year and an 83‐year‐old female who developed lichen planus pemphigoides during the course of thyroid‐gland carcinoma.Conclusion Each of these two entities has its own clinical, histological. immunohistological, and immunobiochemical features. Also, the outcomes seem to be different since lesions of bullous lichen planus usually resolve after a rather short period of time: persistent lesions may possibly develop into squamous cell carcinoma. In contrast, lichen planus pemphigoides is likely to be a paraneoplastic disorder.

Lichen planus pemphigoides: Combination therapy with tetracycline and nicotinamide

It has been speculated that lichen planus pemphigoides (LPP) represents the coexistence of lichen planus (LP) and bullous pemphigoid (BP), a variant of LP, a chance coexistence of LP and another autoimmune bullous dermatitis (i.e., dermatitis herpetiformis), or an independent disease. 1-4 Sobel, Miller, and Shatin 4 suggested that destruction of the basement membrane zone (BMZ) in LP may reveal previously unexposed antigens that result in the formation of circulating antibodies. Mora, Nesbitt, and Brantley 1 suggested that linear deposits of immunoglobins and complement (similar to those found in BP) in LPP may actually be an early immunopathologic change in LP, occurring before the appearance of fluorescent colloid bodies.

Ramipril-associated lichen planus pemphigoides

We report the first case of lichen planus pemphigoides (LPP) secondary to ingestion of ramipril, an angiotensin-converting enzyme inhibitor. Clinical, histological and immunofluorescent findings were all consistent with a diagnosis of LPP. Linear basement membrane zone (BMZ) staining with IgG and C3 was only seen at the roof of split-skin preparations and circulating autoantibody to the BMZ was present at a titre of 1/100. Controlled immunoblotting of epidermal extracts detected the bullous pemphigoid antigens of 230 and 180 kDa.

Lichen planus pemphigoides: Identification of 180 kd hemidesmosome antigen

We describe a man with lichen planus pemphigoides. Direct immunofluorescence studies of peribullous skin showed linear deposition of IgG and C3 in the basement membrane zone. Indirect immunofluorescence studies disclosed circulating anti-basement membrane zone antibodies. Immunoelectron microscopy demonstrated binding of antibodies to the hemidesmosomes and lamina lucida. The patient's serum defined only the minor bullous pemphigoid antigen with a molecular weight of 180 kd. These findings suggest the coexistence of lichen planus and bullous pemphigoid in lichen planus pemphigoides.

Paraneoplastischer Pemphigus

Paraneoplastic pemphigus (PP) is a newly reported autoimmune disease, which is always associated with an underlying neoplasia. It is characterized by painful mucosal erosions and ulcerations, and by polymorphous skin lesions reminiscent of erythema multiforme, pemphigus vulgaris and lichen planus pemphigoides. These patients have autoantibodies that bind to a distinct complex of epidermal proteins, including desmoplakin I (250 kD), major bullous pemphigoid antigen (230 kD), desmoplakin II (210 kD) and a 190-kD and a 170-kD protein, neither of which has yet been further characterized. Histological findings include acantholysis, epidermal cell necrosis, vacuolar interface changes and, sometimes, lichenoid infiltrates of the upper dermis. Direct immunofluorescence shows an intercellular deposition of immunoreactants in the epidermis and granular deposits at the dermal-epidermal junction. Indirect immunofluorescence with rodent bladder as substrate shows an intercellular pattern. This method is the most convenient and cost-effective method of screening for PP, since antigens of both pemphigus vulgaris and pemphigus foliaceus are not expressed in this tissue.

Paraneoplastic Pemphigus Presenting as a Lichen Planus Pemphigoides—like Eruption

Paraneoplastic pemphigus refers to a specific disease entity in which neoplasia is associated with severe mucosal ulceration and polymorphous cutaneous eruptions, sometimes resembling erythema multiforme. Direct immunofluorescence and indirect immunofluorescence on standard substrates are similar to pemphigus vulgaris; however, paraneoplastic pemphigus serum uniquely binds to all epithelia and has distinctive immunoprecipitation characteristics. A 63-year-old woman with chronic lymphocytic leukemia developed a severe, mucocutaneous, vesiculobullous eruption with the clinical and histopathologic characteristics of lichen planus pemphigoides evolving into a Stevens-Johnson-like presentation and the immunofluorescence findings of pemphigus vulgaris. Evaluation of her serum confirmed the presence of autoantibodies specific for paraneoplastic pemphigus by indirect immunofluorescence and immunoprecipitation criteria. The spectrum of paraneoplastic pemphigus must now be expanded to include cases that present clinically and histologically as a lichen planus pemphigoides-like eruption.

Oral and cutaneous lichen planus pemphigoides

Lichen planus pemphigoides is a rare bullous disorder characterized by tense bullae on lichen planus lesions and on clinically uninvolved skin. A diagnosis of lichen planus pemphigoides is made on the basis of clinical, histologic, and immunopathologic evaluation. We describe a patient who had lichen planus pemphigoides of the skin and oral mucosa and briefly review the literature on this uncommon entity.

Lichen planus pemphigoides in a 10-year-old girl

Lichen planus pemphigoides in a 10-year-old girl

Lichen planus pemphigoides: its relationship to bullous pemphigoid

Clinical and immunopathological studies of three patients with lichen planus pemphigoides (LPP) were carried out to investigate the relationship between LPP and bullous pemphigoid (BP) and to determine whether the antigen in LPP is the classical BP antigen. LPP is usually considered to be the coexistence of lichen planus with BP. The bullae in LPP were subepidermal and indistinguishable from BP. Indirect immunofluorescence demonstrated antibody binding to the epidermal surface of 1 M NaCl-split skin and mucosae, as in BP. The tissue distribution of the LPP antigen mirrored the distribution of BP in stratified squamous epithelia but was absent from transitional epithelia (pig bladder). Immunoelectron microscopy, both direct (two cases) and indirect (one case), showed binding to the lamina lucida as with BP antigen. Western blotting of epidermal extracts using the patients' sera showed that instead of reacting with the classical bullous pemphigoid antigen (220 kDa in our series), the antisera reacted with a unique band of 200 kDa in addition to the band of 180 kDa found as a minor antigen in bullous pemphigoid, but more commonly in pemphigoid gestationis. The relationship between these antigens awaits molecular characterization. These findings suggest that the target antigen in LPP may be unique.

Lichen planus pemphigoides

Lichen planus pemphigoides is a rare condition characterized by blisters arising on normal or erythematous skin in a patient with concurrent lichen planus. It must be distinguished from bullous lichen planus, in which, as a consequence of severe basal cell hydropic degeneration, blisters arise within lichenoid papules or plaques. We present a clinicopathological study of nine cases of lichen planus pemphigoides, and report histological, immunofluorescent, ultrastructural and immuno-electronmicroscopical observations. We distinguish lichen planus pemphigoides from bullous lichen planus and consider the differential diagnosis. We propose that lichen planus pemphigoides does not represent a homogeneous condition: it may represent a number of bullous dermatoses that develop as a consequence of exposure of different basement membrane antigens following severe damage to the epidermal basement membrane as part of the lichenoid inflammatory process.

Deposition of C3, C9 neoantigen and vitronectin (S-protein of complement) in lichen planus pemphigoides

We have compared the distribution of C3, C9 neoantigen (C9n) and vitronectin at the dermoepidermal junction in lichen planus pemphigoides with that in bullous pemphigoid. Eight out of 30 biopsies from patients with lichenoid lesions had linear C3 deposition at the basement membrane zone (BMZ); four of these patients had bullae and fulfilled the criteria for lichen planus pemphigoides. C9n immunoreactivity was detected as a linear or an intermittent linear/granular band at the BMZ only in these four patients, suggesting a role for the membrane attack complex of complement (MAC) in the pathogenesis of blister formation in lichen planus pemphigoides. Faint linear deposition of vitronectin, in addition to C9n, at the BMZ was seen in two of the four cases of lichen planus pemphigoides and three of six cases of bullous pemphigoid. This suggests that vitronectin may be deposited in association with C9n not only as part of the non-lytic SC5b-9 complex, but also as a regulatory step following the lytic action of MAC. A regulatory function for vitronectin in limiting tissue damage following activation of MAC is supported by our finding of a heavy deposition of vitronectin in association with C9n in a lichen planus pemphigoides patient in whom bulla formation had ceased.

Ultrastructural localization of the binding sites of anti-BMZ antibodies in lichen planus pemphigoides

Ultrastructural localization of the binding sites of anti-BMZ antibodies in lichen planus pemphigoides

Lichen planus pemphigoides: Case report and results of immunofluorescence and immunoelectron microscopic study

A Japanese woman with lichen planus pemphigoides is reported. Immunologic characteristics of lichen planus pemphigoides antigen in the patient were investigated by indirect immunofluorescence and compared with those of bullous pemphigoid antigen or epidermolysis bullosa acquisita antigen. Ultrastructural localization of lichen planus pemphigoides antigen was studied with the use of immunoelectron microscopic techniques. Lichen planus pemphigoides antigen showed localization similar to that of bullous pemphigoid an tigen but different from that of epidermolysis bullosa acquisita antigen. The antigenic stability of lichen planus pemphigoides antigen was different from that of bullous pemphigoid antigen or epidermolysis bullosa acquisita antigen. Thus this study demonstrates that lichen planus pemphigoides antigen is different from bullous pemphigoid antigen.

Lichen planus pemphigoides

Lichen planus pemphigoides is a rare disease in which blistering occurs in association with lesions of classical lichen planus. We describe a patient with lichen planus pemphigoides and discuss the evidence for there being one disease rather than the coincidence of bullous pemphigoid and lichen planus.

Lichen Planus Pemphigoides With Features of Lichen Planus and Pemphigus Vulgaris

To the Editor.— Since the establishment of immunofluorescence techniques, there has ceased to be a controversy concerning the nosologic relation of lichen planus pemphigoides. The coexistence of lichen planus with a primary bullous disorder, once thought to represent a form of bullous lichen planus, was shown in most cases to have the immunologic characteristics of bullous pemphigoid. 1 We describe a patient suffering from a rare variant of lichen planus pemphigoides, having the histologic features of both lichen planus and pemphigus vulgaris, confirmed by immunofluorescence. Report of a Case.— A 76-year-old woman was admitted to our department with a 2-month history of a generalized eruption consisting of hyperpigmented papules, which was followed one month later by the appearance of loose vesicles and bullae. Four years previously, she had received irradiation with roentgen rays with combination chemotherapy for intra-abdominal lymphoma. On examination, the patient was found to be febrile

Lichen planus pemphigoides with features of lichen planus and pemphigus vulgaris

<h3>To the Editor.—</h3> Since the establishment of immunofluorescence techniques, there has ceased to be a controversy concerning the nosologic relation of lichen planus pemphigoides. The coexistence of lichen planus with a primary bullous disorder, once thought to represent a form of bullous lichen planus, was shown in most cases to have the immunologic characteristics of bullous pemphigoid.¹ We describe a patient suffering from a rare variant of lichen planus pemphigoides, having the histologic features of both lichen planus and pemphigus vulgaris, confirmed by immunofluorescence. <h3>Report of a Case.—</h3> A 76-year-old woman was admitted to our department with a 2-month history of a generalized eruption consisting of hyperpigmented papules, which was followed one month later by the appearance of loose vesicles and bullae. Four years previously, she had received irradiation with roentgen rays with combination chemotherapy for intra-abdominal lymphoma. On examination, the patient was found to be febrile

Ultrastructural localization of the binding sites of antibasement membrane zone antibodies in lichen planus pemphigoides on 1.0 M sodium chloride-separated skin.

Etude en immunofluorescence indirecte, en microscopie optique et electronique d'echantillons de peau provenant de sujets sains, de sujets atteints de pemphigoide bulleuse et de sujets presentant un lichen plan pemphigoide. Le but de cette etude est de localiser les sites de liaison des anticorps du lichen plan pemphigoide et de determiner les proprietes des antigenes de cette pathologie

Lichen planus pemphigoides: Report of a case with oral lesions

Lichen planus pemphigoides is a rare condition characterized by the coexistence of lichen planus and bullous pemphigoid. Oral lesions have been reported but have not been studied immunopathologically. We describe a 59-year-old white man with cutaneous and oral lesions of lichen planus pemphigoides. Biopsies were done on these lesions, and the specimens were examined by routine light microscopy and immunofluorescent techniques. Fine keratotic striae on the anterior buccal mucosa were clinically consistent with oral lichen planus. Perilesional tissue associated with ulceration of the posterior buccal mucosa showed histologic and immunopathologic changes consistent with bullous pemphigoid.