Skin antibodies in bone marrow transplanted patients

Direct and indirect immunofluorescent studies were performed in seventeen bone marrow transplanted patients (BMT). No immunoglobulins were found in the maculo papular lesions of the early graft versus host reaction. Globular deposits of IgM and complement were found in the skin specimens from lichen planus like eruptions; these were very similar to those found in idiopathic lichen planus. Circulating epidermal cytoplasmic antibodies (ECA) were detected in 15 of 17 (88%) patients as shown by a longitudinal study of 118 sera. This frequency was significantly higher than in other groups in this study (133 subjects). ECA did not correlate with a graft versus host reaction. It is postulated that they develop as a consequence of chronic skin damage in subjects with disorders of the immune system which stimulate the production of numerous antibodies.     

Bullous lichen planus: diagnosis by indirect immunofluorescence and treatment with dapsone

Bullous lichen planus may be confused with other subepidermal bullous dermatoses, especially if bullae arise on normal-appearing skin. We present two patients who were originally thought, on the basis of clinical and histopathologic criteria, to have bullous pemphigoid. However, results of standard indirect and direct immunofluorescence assays did not support the diagnosis of bullous pemphigoid. Further investigation using the indirect autologous immunofluorescence assay revealed deposits of immunoglobulins in the stratum granulosum. Moreover, the indirect allogeneic immunofluorescence assay (using papular lichen planus lesional substrates from different patients) was positive for the same pattern, confirming the diagnosis of bullous lichen planus. Bullous lichen planus with bullous pemphigoid-like histologic features can be differentiated from bullous pemphigoid on the basis of the indirect autologous and allogeneic immunofluorescence assays for circulating antigranulosum antibodies. Both patients were treated with systemic corticosteroids. One patient went into remission with this therapy alone; the other patient had a favorable response when dapsone was given with systemic corticosteroids. The suggestion that there is a subset of bullous lichen planus with bullous pemphigoid-like histologic features that responds to dapsone needs to be explored.     

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.     

Childhood Lichen Planus Pemphigoides: A Case Report and Review of the Literature

Abstract:  Lichen planus pemphigoides is a rare autoimmune blistering disease that is characterized by evolution of vesico-bullous skin lesions in patients with active lichen planus. We describe a case of lichen planus pemphigoides in a 6-year-old boy and review the clinical and immunopathologic features of all reported cases of pediatric lichen planus pemphigoides. The mean age at onset of childhood lichen planus pemphigoides is 12 years with a male to female ratio of 3:1 and a mean lag-time between lichen planus and the development of lichen planus pemphigoides of 7.9 weeks. Vesiculo-bullous lesions were found on the extremities in all patients and there was palmoplantar involvement in about half of the cases. Direct and indirect immunofluorescence features were similar to those reported in adults. One patient had Western immunoblot data revealing antigens of 180, 230, and 200 kDa. Immunoelectron microscopy in two cases showed localization of immune deposition different from that in bullous pemphigoid. We found that topical corticosteroids or oral dapsone caused resolution of lichen planus pemphigoides without known relapse of blistering in four cases, suggesting that it might be possible to reserve oral corticosteroids as a second line of therapy in children with lichen planus pemphigoides.     

Lichen nitidus: electron microscopic and immunofluorescence studies

Skin biopsies from a patient suffering from lichen nitidus were studied by electron microscopy and immunofluorescence studies. Complete disintegration of the stratum basale in the central area of the lesion and signs of acantholysis in the border area were found. In the central part of the lesion the dermo-epidermal junction was severely damaged in most places. In the dermal infiltrate, macrophages and lymphocytes were found with a considerable representation of Sézary cells, not previously reported in lichen nitidus. The ultrastructural changes are identical with those found in lichen planus. No in vivo bound immunoglobulins, fibrinogen, or complement C3 were found.     

Griseofulvin therapy of lichen planus

Thirty-one patients who had lichen planus treated with griseofulvin were randomly selected for review from a group of patients with lichen planus seen at the Mayo Clinic between January 1976 and June 1980; two patients were excluded because of lack of adequate follow-up. Of the 11 patients with only oral lesions, 6 showed a marked improvement or complete remission. Of the 18 patients with lichen planus involving one or more sites with or without oral lesions, 15 had cutaneous lesions. Three of the 15 had improvement of their cutaneous lesions; however, 1 of the 3 continued to develop new lesions, although old ones were improving. In patients with recalcitrant, symptomatic oral lichen planus, a trial of griseofulvin would seem justified. Success in patients with cutaneous lichen planus is less likely; however, griseofulvin may afford relief in selected patients. This study indicates that further prospective studies are needed to clarify the efficacy of griseofulvin in lichen planus.     

IMMUNOFLUORESCENCE STUDY OF DRUG-INDUCED LICHEN PLANUS-LIKE LESIONS

Using an immunofluorescence technique, we investigated lesions of drug-induced lichen planus-like lesions from patients. In addition, a comparative study of idiopathic lichen planus from thirteen patients was made. Deposits of fibrinogen along the epidermal basement zone, IgM, and less often IgA, IgG, C₁q and C3 on the colloid bodies were found in drug-induced lichen planus-like lesions. Similar deposits of immunoglobulins and complements were noted in 2 of 3 lesions developed by successful provocation tests. Deposits of fibrinogen were also demonstrated in all 3 lesions. However, no circulating antibodies directed toward the epidermal basement zone or other skin components were detected. No elevation of circulating immune complex levels was noted. Immunofluorescence findings in drug-induced lichen planus-like lesions were essentially identical to those in idiopathic lichen planus. This indicates that both conditions share a common disease process.     

Primary Cutaneous Amyloidosis: Identically Distributed Lesions in Identical Twins

Male HLA-identical twins with numerous congenital abnormalities were discovered to have lichen amyloidosus in similar distribution on their chests and abdomens. Pruritus was absent in both. The finding of identical distribution of nonpruritic lesions was previously reported in familial lichen amyloidosus. This suggests that a subset of these patients have a nevoid condition in which a circumscribed patch of keratinocytes undergoes filamentous degeneration and may appear in identical distribution in family members in the absence of pruritus.     

Immunohistochemistry in lichen planus.

An immunohistochemical study has been undertaken on 25 biopsy specmens taken from lichen planus lesions, using antisera against human fibrin, immunoglobulins IgG, IgA, IgM and C3 (B1C/B1A) complement component. The findings of the present research are discussed and evaluated in relation to the problem of the etiopathogenesis of the disease.     

The period prevalence of oral lichen planus in a cohort of patients with vulvar lichen sclerosus

Background Lichen sclerosus and lichen planus are chronic inflammatory mucocutaneous disorders that may coexist. Objective The aim of this study was to estimate the period prevalence of oral lichen planus in a cohort of patients with vulvar lichen sclerosus and to document their clinical characteristics. Methods We report a series of cases of vulvar lichen sclerosus presenting to two dermatologist‐led vulvar clinics in Oxfordshire, England between 1997 and 2007 with coexistent clinical signs of oral lichen planus. Results Thirteen cases with coexistent vulvar lichen sclerosus and oral lichen planus were identified, of which five had oral biopsies. Four oral biopsies showed histological features consistent with lichen planus. One oral biopsy was not diagnostic but compatible with oral lichen planus. No cases of oral lichen sclerosus were identified. The period prevalence of oral lichen planus was 6 per 1000 cases of vulvar lichen sclerosus. Conclusion The period prevalence of oral lichen planus in women with vulvar lichen sclerosus (0.6%) is similar to that reported for oral lichen planus in the general population (1–2%).     

Evaluation of clinical types of cutaneous lichen planus in anti-hepatitis C virus seronegative and seropositive Nigerian patients

BACKGROUND: The presentation of oral lichen planus in anti-hepatitis C virus (HCV) seropositive and seronegative patients was previously evaluated, and the keratotic form of oral lichen planus was found to be more prevalent in anti-HCV seropositive patients. This study evaluated the presentation of cutaneous lichen planus in anti-HCV seropositive and seronegative Nigerians. METHODS: Fifty-seven Nigerians with cutaneous lichen planus were carefully examined to determine the form of lichen planus present. All were screened for the presence of anti-HCV by second-generation enzyme-linked immunosorbent assay (ELISA) and grouped as anti-HCV seropositive or anti-HCV seronegative patients. RESULTS: Nine patients were anti-HCV positive. Seven of these seropositive patients had hypertrophic lichen planus. CONCLUSION: Hypertrophic lichen planus in Nigerians is more prevalent with HCV infection.     

Lichen amyloidosus associated with Kimura's disease: successful treatment with cyclosporine

We describe here a case of a 33-year-old man who had lichen amyloidosus associated with Kimura's disease. In this case, treatment with cyclosporine dramatically improved the lesions of both Kimura's disease and lichen amyloidosus. Although Kimura's disease and lichen amyloidosus are both rare distinct entities, to our knowledge, 11 cases of association of Kimura's disease and lichen amyloidosus have been described previously.     

Mixed Lichen Planus-Lupus Erythematosus Disease

Two patients are reported with clinical symptoms of systemic lupus erythematosus associated with centrally ulcerated hypertrophic plaques and lip lesions. Histological findings from various sites were features of both lupus erythematosus and lichen planus. An increase in acid mucopolysaccharides and a thickened basement membrane which were present in some biopsies were interpreted as lupus erythematosus. The deposition of immunoglobulins and fibrinogen seemed to favor lupus erythematosus but these findings may also be seen in lichen planus. Four possible interpretations of the findings are discussed. The term "mixed lichen planus--lupus erythematosus disease" may be appropriate for these patients.     

Co-existence of lichen amyloidosus and angiolymphoid hyperplasia with eosinophilia

Summary Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular tumour. It presents with small, dull red papules or nodules usually on the ears and preauricular areas and only 20% of lesions are multiple. We report a case of multiple scattered lesions of ALHE in a patient who subsequently developed lichen amyloidosus. Cases of lichen amyloidosus in association with Kimura's disease have been reported previously, but there are no reports of lichen amyloidosus with ALHE. The coexistence of these two conditions implies that ALHE is an inflammatory disorder, as an inflammatory process resulting in basal layer damage is necessary for the occurrence of lichen amyloidosus.     

Immunofluorescence studies on complement components in lichen amyloidosus

Immunofluorescence studies were carried out in 7 cases of lichen amyloidosus, chiefly to detect deposition of complement components in the cutaneous lesions. Examination of skin biopsy specimens revealed deposition of Clq, C3, C9 and IgM in all the patients studied. Complement and immunoglobulin levels by assays of simultaneously obtained serum samples were found to be almost within the normal limits.     

A study on the association with hepatitis B and hepatitis C in 1557 patients with lichen planus

Background Previous reports have demonstrated contradicting results on the association between lichen planus and hepatitis. Objectives The aim of this study was to investigate the association between lichen planus and viral hepatitis. Methods Patients with lichen planus were compared with controls regarding the prevalence of viral hepatitis in a case‐control study using logistic multivariate regression models. The study was performed utilizing the medical database of Clalit Health Services. Results The study included 1557 lichen planus patients over the age of 20 years and 3115 age‐ and gender‐matched controls. The prevalence of hepatitis C in patients with lichen planus was higher than that in the control group (1.9%, 0.4% respectively, P < 0.001). In a multivariate analysis, lichen planus was associated with hepatitis C (OR 4.19, 95% CI 2.21; 7.93). The prevalence of hepatitis B in patients with lichen planus was similar to that in the control group (0.9%, 0.5% respectively, P = 0.12). A multivariate analysis revealed that lichen planus was not associated with hepatitis B (OR 1.69, 95% CI 0.82; 3.47). Conclusion Lichen planus is associated with hepatitis C but not with hepatitis B. Physicians who care for patients with lichen planus should consider screening patients with lichen planus for hepatitis C.     

Primary biliary cirrhosis and lichen planus

Lichen planus and primary biliary cirrhosis were seen in twenty-four patients. In seventeen patients, the cutaneous eruption followed the administration of D-penicillamine. In seven patients, lichen planus developed unrelated to therapy. Three of the latter group of patients were treated with D-penicillamine and had subsequent relapse or exacerbation of their preexisting lichen planus. The presence of lichen planus in patients with primary biliary cirrhosis and the propensity to develop this type of eruption while on D-penicillamine therapy are consistent with a graft-versus-host pathogenesis of primary biliary cirrhosis. Preexisting lichen planus should be regarded as a relative contraindication to the use of D-penicillamine in patients with primary biliary cirrhosis.     

Antikeratin autoantibodies in the amyloid deposits of lichen amyloidosus and macular amyloidosis

Summary In order to characterize immunoglobulins found on amyloid deposits of lichen amyloidosus and macular amyloidosis, an elution from cryostat sections was performed with citrate buffer, glycine buffer, NaCl, and PBS. Resulting eluates (mainly IgG) were examined with dot immunoblotting and SDS-PAGE immunoblotting and were found to react with the human epidermal keratin of 50 and 67 kD. Antikeratin autoantibody activities in normal murine and human sera were examined using a dot immunoblotting assay. In murine sera, titers of IgG and IgM autoantibodies were higher in older mice. The human cord blood showed significantly lower IgM autoantibody titers, whereas IgG antibody titers showed no significant differences from adults' sera, probably due to the permeability of IgG through the placental barrier. A stronger antibody activity in older individuals was thought to be due to the repeated exposures to keratin proteins derived from apoptotic keratinocytes. Sera from lichen amyloidosus and macular amyloidosis patients did not show any difference from normal controls in their antikeratin titers. It was concluded that the patients with lichenoid or macular amyloidosis are capable of producing a normal level of antikeratin autoantibodies. However, the removal of opsonized keratin-type amyloid from the skin is slow or deficient due to as yet unknown factors.Part of this study was reported on May 5, 1987 at the 48th Annual Meeting of the Society for Investigative Dermatology at San Diego, CA, USA     

Unusual variant of lupus erythematosus or lichen planus. Clinical, histopathologic, and immunofluorescent studies.

Eleven patients with a skin disorder in which clinical, histopathologic, and immunofluorescent findings showed overlap features of both lupus erythematosus (LE) and lichen planus (LP) were observed for several years. Clinical lesions were extremely long-term and consisted primarily of livid red to violaceous atrophic patches and plaques, most common on acral aspects of the extremities. Nails were also commonly involved, often showing anonychia. Histologic changes combined cell-rich and cell-poor lichenoid patterns in the papillary dermis, suggesting both LP and LE. The major immunofluorescent finding in all patients was the presence of ovoid bodies at the dermal-epidermal (D-E) junction and in the upper dermis. Most patients showed both a linear arrangement and a clustering pattern of these bodies. Four patients also had coexistant but poorly developed linear deposits of immunoglobulins and complement at the D-E junction.     

Light and electron microscopy of primary localized cutaneous amyloidosis

The two polar types of primary cutaneous amyloidosis are characterized by different alterations of the epidermis, i.e., epidermal hyperplasia and hyperkeratosis in lichen amyloidosus, hyperpigmentation without any essential epidermal changes in macular pigmented amyloidosis. Biopsies from six patients suffering from lichen amyloidosus and macular pigmented amyloidosis were examined light- and electron-microscopically to provide any information that could account for the different epidermal behavior in these two polar forms of primary cutaneous amyloidosis. No ultrastructural alterations were found that could be regarded as pathognomonic for one or the other form. Although in lichen amyloidosus alterations on keratinocytes seemed to be more pronounced than in macular pigmented amyloidosis, which, in contrast, shows stronger alterations of the basal membrane and the pigmentary system, these findings do not satisfactorily elucidate the histological and clinical differences between these most frequently occurring representatives of primary cutaneous amyloidosis.