Cases reported "Lichen Planus"

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1/29. Demonstration of antibody to 230 kDa bullous pemphigoid antigen in lichen planus-like keratosis.

    We describe a 67-year-old man with lichen planus-like keratosis associated with anti-230 kDa bullous pemphigoid antigen (BPAG1) autoantibody. The patient had noticed solitary dark brown macule more than 6 years previously on his left chest. Histological findings showed hypergranulosis, irregular acanthosis, liquefaction degeneration of basal cells, band-like infiltration of lymphocytes at the subepidermal portion, and a cleft at the basement membrane zone (BMZ), resulting in the formation of subepidermal blisters. Direct immunofluorescence findings of perilesional skin showed a linear deposition of IgG at BMZ. On indirect immunofluorescent study using normal human skin, circulating IgG autoantibody to BMZ was present in the patient's serum at a titer of 1:80. The antigen located on the epidermal site of normal skin split by 1M NaCl was reacted with the patient's serum. Immunoblot analysis using epidermal extracts demonstrated the presence of IgG antibody directed to BPAG1 in the patient's serum. These observations suggest that the presence of an antibody to BPAG1 could be caused by the damage of basal cells following lichen planus-like keratosis.
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2/29. IgG autoantibodies from a lichen planus pemphigoides patient recognize the NC16A domain of the bullous pemphigoid antigen 180.

    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.
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3/29. lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen).

    We describe a 75-year-old patient with pruritic papules on her trunk and extremities, typical of lichen planus, who later experienced subepidermal blisters. These clinical features are consistent with lichen planus pemphigoides. Immunofluorescence of perilesional skin showed linear deposits of C3 along the dermoepidermal junction. Circulating IgG autoantibodies were found to be directed against an epidermal component of the dermoepidermal junction because the patient's serum labeled the epidermal side of 1 mol/L NaCl-split skin. The patient's IgG autoantibodies were directed exclusively against the 180 kd bullous pemphigoid antigen (BPAg2, type XVII collagen) detected in human keratinocyte lysate by Western blot assay. No reactivity was found against the 230 kd bullous pemphigoid antigen, type VII collagen, or the laminin-5 subunits. This study demonstrates that BPAg2 is recognized, not only by bullous pemphigoid sera, but also by lichen planus pemphigoides sera. Our findings attest to the similarity of immunopathology in these two subepidermal blistering skin diseases.
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4/29. lichen planus pemphigoides with circulating autoantibodies against 200 and 180 kDa epidermal antigens.

    We describe a case of lichen planus pemphigoides with circulating autoantibodies against 200 and 180 kDa epidermal antigens. A 24-year-old man presented pruritic erythematous patches with occasional central blister formation on the face, neck, and trunk and erythematous hyperkeratotic patches on the elbows and feet for 10 months. Histopathological findings of the neck and plantar lesions were consistent with lichen planus. Direct immunofluorescence studies from both lesions showed a linear deposition of IgG and C3 and cytoid bodies along the basement membrane zone. Indirect immunofluorescence studies demonstrated circulating IgG antibodies deposited on the epidermal side of salt-split skin. Immunoblot analysis using epidermal extract showed that the patient's serum reacted with 180 kDa bullous pemphigoid antigen (BPAG2) and a 200 kDa antigen. The cutaneous lesions almost cleared with a low dose of prednisolone and acitretin.
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5/29. Lichenoid dermatitis in paraneoplastic pemphigus: a pathogenic trigger of epitope spreading?

    BACKGROUND: In select cases, lichen planus has been observed to be a paraneoplastic condition sometimes associated with paraneoplastic pemphigus, a disease featuring autoantibodies directed against plakin proteins, desmogleins 3 and 1, and a still uncharacterized 170-kd antigen. Epitope spreading describes the phenomenon where underlying chronic inflammation leads to the sequential recognition of new epitopes on self-proteins over time. OBSERVATIONS: Five of 6 patients diagnosed as having paraneoplastic pemphigus had concomitant clinical and histological features of lichen planus. In 1 patient, results of the initial indirect immunofluorescence on rat bladder were negative and only 2 of the 5 antigens were identified by immunoprecipitation. After 1 year of worsening disease, repeated testing confirmed the presence of antibodies directed against all 6 of the implicated antigens, supportive of our hypothesis that epitope spreading may occur in paraneoplastic pemphigus. CONCLUSIONS: lichenoid eruptions may predispose to an early evolutionary stage of paraneoplastic pemphigus. Cell-mediated autoimmunity at the dermoepidermal junction may promote the exposure of self-antigens and the development of subsequent and progressive humoral autoimmunity. As such, paraneoplastic pemphigus may demonstrate epitope spreading in a human, humoral-mediated autoimmune disease.
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6/29. Previous tuberculosis, hepatitis c virus and lichen planus. A report of 10 cases, a causal or casual link?

    We report 10 cases of lichen planus (LP) and chronic liver disease linked to HCV. The mean age was 63.4 /- 5.1 years (range 51-73), five were female; six patients had an established cirrhosis of the liver, as shown by either a liver biopsy or the ultrasonographic and biohumoral evidence. The remaining four patients had chronic hepatitis. Histological examination confirmed the presence of LP: the localization of the dermatosis was restricted to the skin in four patients, to the mucous membranes in five (4 atrophic erosive and one erosive) while the remaining had mucous-cutaneous localization. A type II cryoglobulinemia was demonstrated in two and a type III in one of the patients, while no one had otherwise circulating autoantibodies (anti-nuclear, anti-smooth muscle, anti-liver kidney microsomal type 1 and anti-mitochondrial antigens) such as other etiological factors of liver disease. In six of the patients the history was positive for previous mycobacterium tuberculosis infection. In clinical practice the patients with chronic liver disease and HCV infection can also suffer from severe extrahepatic manifestations, including lichen planus.
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7/29. Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides-like eruption. A case report and review of literature.

    A 50-year-old man presented with severe mucosal erosions of the lips, oral cavity and perianal area, a lichen planus-like eruption on the trunk and extremities and scaly plaques of the palms and soles. The clinical impression was of Stevens--Johnson syndrome, or paraneoplastic pemphigus (PNP). Histopathology revealed vacuolar interface and lichenoid dermatitis with dyskeratosis and suprabasal acantholytic vesiculation. Direct immunofluorescence showed deposition of IgG in the intercellular space and linear deposition of C3 along the basal membrane zone. Indirect immunofluorescence revealed circulating IgG with intercellular staining of the epithelium of rat urinary bladder. Western blotting demonstrated bands of 250- and 230-kDa antigens. The clinical, histological and immunological features were consistent with the lichen planus pemphigoides variant of PNP. A retroperitoneal hyaline-vascular Castleman's disease was detected and excised. The skin lesions worsened initially after tumour resection but improved gradually, leaving extensive melanosis after cyclosporin and mycophenolate mofetil treatment.
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8/29. Coexistence of Riehl's melanosis and lichen planus.

    A 61-year-old man presented with purple-red papules and plaques. These lesions on the nape and dorsal aspects of both hands displayed the histological findings characteristic of lichen planus. Riehl's melanosis, which is synonymous with pigmented contact dermatitis, was present on the forehead and bald scalp. Riehl's melanosis typically appears on the face and is induced by cosmetics. Causative factors in this case, however, remained undefined. The coexistence of lichen planus and Riehl's melanosis at separate sites in a single patient suggests that the two diseases represent manifestations of specific immune responses characteristic of lichenoid tissue reaction and situate in the same spectrum. The differences in the skin manifestations of lichen planus and Riehl's melanosis might depend on the severity of immune responses against intrinsic or extrinsic antigens. In addition, the skin manifestations of lichenoid tissue reaction might depend on the disposition of the lesion sites.
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9/29. PUVA-induced lichen planus.

    A 28-year-old male with histologically proven psoriasis vulgaris was administered oral 8-methoxypsoralen UVA therapy (PUVA). The plaques of psoriasis gradually responded to treatment, however numerous pruritic, violaceous papules over the right forearm and left thigh developed 5 months after starting PUVA (45 sittings with a cumulative dose of 156 J/cm(2)). Histopathologic examination of these lesions was compatible with the diagnosis of lichen planus (LP). On stopping puva therapy, the lesions subsided in one month. This case emphasizes the hypothesis that cell injury caused by puva therapy could expose some sequestered antigens to autoreactive lymphocytes and induce lichen planus.
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10/29. lichen planus pemphigoides evolving into pemphigoid nodularis.

    lichen planus pemphigoides (LPP) and pemphigoid nodularis are rare clinical variants of bullous pemphigoid (BP), which are characterized by histological findings of lichen planus (LP) and nodular prurigo, respectively, and the finding of linear deposits of IgG and/or C3 at the basement membrane zone in perilesional skin. In both cases bullae may arise at the site of pre-existing LP-like or nodular prurigo-like eruptions, and clinically uninvolved skin. The disease spectrum of LPP and pemphigoid nodularis differs from that of classical BP phenotype, and their presentations are often indolent. LPP may predominantly affect a younger age group and is responsive to standard treatments used in acquired autoimmune bullous diseases, while pemphigoid nodularis is more common in elderly women and is relatively resistant to therapy. We describe a patient who had LPP for nearly two decades and subsequently developed a nodular eruption with a concurrently detected antibullous pemphigoid antigen 2 (BP180) autoantibody. His overall clinicopathological features were indicative of LPP evolving into another BP variant, pemphigoid nodularis.
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