Isolated lichen planus of the nails associated with primary biliary cirrhosis

Lichen planus is a relatively common inflammatory dermatosis with characteristic lesions affecting the skin, nails and mucous membranes. Nail changes are present in approximately 10% of patients and can occasionally occur in the absence of skin involvement. The association of lichen planus with primary biliary cirrhosis is now well recognized. We report the first case of lichen planus confined to the nails in a patient with primary biliary cirrhosis.     

Lichen planus and primary biliary cirrhosis

The association of lichen planus and primary biliary cirrhosis in five patients is reported. The coexistence of the two diseases is probably more than coincidental and may be due to the fact that both conditions are based on an alteration of immune mechanisms. There is great similarity between primary biliary cirrhosis and chronic graft-versus-host disease and a common pathogenesis for the two entities has been postulated. Since lichen planus is one of the most common manifestations of graft-versus-host disease, its association with primary biliary cirrhosis is of significance, although not unexpected.     

Successful treatment of hypertrophic lichen planus with betamethasone under occlusion and TCA‐peelings

Hypertrophic lichen planus (HLP) is a variant of lichen planus characterized by marked epidermal hyperplasia and severe pruritus. We present a case of a female patient with HLP and concomitant primary biliary cirrhosis, which responded to topical therapy with betamethasone under occlusion and TCA‐peelings.     

Lichen-hepatitis syndrome. General review apropos of a case

Recently some evidence has accumulated indicating that lichen ruber planus, especially in its erosive variety, may be an important indicant of chronic cirrhogenic hepatitis, either a chronic active hepatitis or a primary biliary cirrhosis. Usually the cutaneous lesions precede the obvious clinical features of liver disease by months or years and the erosive quality of the lesions usually indicates the presence of hepatic cirrhosis. A case is reported herein which had so typical features of both lichen planus and erosivus and chronic active hepatitis to be regarded as representative of this association. In Italy, possibly due to the high prevalence of hepatitis B virus infection, the lichen planus patients seem to have a higher probability to develop a chronic active hepatitis, whereas those in Great Britain and USA, for genetic reasons perhaps, seem to associate with primary biliary cirrhosis. The distinction of the two hepatic diseases is difficult in the earliest stage, but it is very important, because their treatments are quite different. The association lichen planus-chronic active hepatitis appears not to be fortuitous and its possible pathogenetic mechanism is proposed.     

Lichenoid eruption following hepatitis B vaccination: first North American case report

Lichen planus is often found in association with a variety of underlying conditions. In particular, liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, hepatitis C, and hepatitis B have been implicated in cutaneous lichen planus. Of interest, there is mounting evidence that lichen planus-like eruptions can occur following administration of the hepatitis B vaccine, which has recently become a routine immunization in many parts of the world. We present what we believe to be the first North American case of lichenoid drug eruption associated with the hepatitis B vaccine and provide a brief review of other reported cases of lichenoid eruption seen following hepatitis B vaccination.     

Concurrent oral lichen planus and primary sclerosing cholangitis

Four patients with concomitant oral lichen planus (OLP) and primary sclerosing cholangitis (PSC) are presented. Associations have been made between OLP and chronic liver disease, namely hepatitis C and primary biliary cirrhosis, but the aetiology and commonality between the diseases has yet to be confirmed. An immunological link is currently favoured. PSC may be a further association with OLP, possibly involving the immune system.     

Multiples Autoimmunsyndrom

A female patient presented with acral scleroderma, Sjögren syndrome, antibodies specific for primary biliary cirrhosis and clinical as well as histological features of lichen planus and subacute lupus erythematosus. In addition an euthyroid Hashimoto thyroiditis was found. Her findings correspond to type II of the multiple autoimmune syndrome (MAS) and can be described as an association of Reynolds syndrome and the lupus erythematosus/lichen planus-overlap syndrome.     

Captopril-induced lichen planus pemphigoides with pemphigus-like features. A case report

We report a case of a bullous lichenoid eruption due to the intake of captopril. Clinical, histological, direct immunofluorescence and ultrastructural features were consistent with the diagnosis of lichen planus pemphigoides. In addition, the in vivo immunological study also revealed an intercellular fluorescence, similar to that seen in pemphigus. Complex drug-induced cutaneous reactions have been previously reported with other drugs, especially with D-penicillamine, which bears chemical similarities with captopril. However, such a drug-induced mixed pattern of lichen planus pemphigoides with pemphigus-like features has never been reported.     

Clinical and laboratory presentation of lichen planus patients with chronic liver disease.

A recent case-control study on 577 lichen planus (LP) patients and 1008 controls confirmed that LP patients may significantly associate with a chronic liver disease (CLD) which is independent from drug or alcohol intake and has some connection with hepatitis B virus (HBV) infection. The study, however, failed to define the nature of CLD. This has been investigated through the clinical and laboratory features of 50 patients with LP and impaired liver function tests. Overall, the laboratory signs of cell necrosis prevailed over those of cholestasis and a good relationship with the HBV and HCV infections was found. Ninety percent of patients with LP and CLD had antibodies to one or another of the major viruses involved in infectious hepatitis. No patient had anti-liver kidney microsomal antibodies type 1. Liver biopsies were done in 12 cases and mostly revealed a chronic active hepatitis evolving into cirrhosis. No evident cases of primary biliary cirrhosis were found. It appears that LP associated CLD is post-viral in nature.     

Trioxsalen baths plus UV-A in the treatment of lichen planus and urticaria pigmentosa

Local photochemotherapy with trioxsalen baths and long-wave ultraviolet radiation (UV-A) was used in nineteen patients with lichen planus and five patients with urticaria pigmentosa. Widespread papular lichen planus healed totally in all sixteen patients. Hypertrophic lichen planus was more resistant to therapy; two out of three patients recovered completely and in one the result was good. The average total UV-A dose in papular lichen planus was only 6 J/cm^2. In urticaria pigmentosa good results were gained in all five patients treated with respect to whealing, itching and dermographism, and in one patient the lesions disappeared completely.     

Lichen planus-like lesions caused by penicillamine in primary biliary cirrhosis

Six patients with primary biliary cirrhosis experienced lichen planus-like lesions while taking penicillamine. Oral lesions developed in all patients, and the lichenoid lesions also appeared elsewhere in two patients. Lichen planus-like lesions developed from four to 16 months after penicillamine therapy had been started, and, in four cases, they resolved in one to three months after use of the drug was stopped. Two patients have continued using penicillamine despite adverse cutaneous and mucosal reactions and have continued to experience lichen planus-like lesions intermittently. Other cutaneous reactions associated with penicillamine are reviewed.     

Behandlung des therapieresistenten erosiven,oralen Lichen ruber mit extrakorporaler Photopherese (ECP)

Background: Erosive, oral lichen planus is typically therapy‐resistant. Histologically and immunopathologically there are many similarities between lichen planus and lichenoid graft versus host disease (GvHD). Extracorporeal photopheresis (ECP) therapy has been shown effective in GvHD in several publications; only one study addresses its use in chronic erosive lichen planus. Patients and Methods: Four patients with erosive oral lichen planus were treated. Therapy was performed on two consecutive days (therapy cycle) every two weeks. Following clinical improvement, the therapy intervals were prolonged. Results: In all four patients clinical symptoms and mucosal lesions improved after seven to nine therapy cycles. A temporary worsening occurred in two patients following dental procedures. One of those patients still requires regular ECP therapy. Two patients discontinued therapy following nearly complete remission for other reasons. One patient stopped therapy after 19 cycles of ECP therapy and has remained in complete remission for 9 months. No side effects were seen during treatment. Conclusions: Extracorporeal photopheresis is an effective therapeutic option for the treatment of erosive oral lichen planus, especially due to the lack of side effects in contrast to other established therapies. Adjunctive topical treatment is also required.     

Lichen planus and cicatricial conjunctivitis: disease course and response to therapy of 11 patients

Background Lichen planus is an autoimmune, inflammatory dermatosis of unknown cause that affects the skin and mucous membranes. Objective The aim of this study was to report the clinical features and response to therapy in a series of patients with ocular lichen planus. Methods A retrospective chart review was performed to identify patients with ocular lichen planus. Information about clinical presentation, treatment, and therapeutic response was extracted from the medical records. Results Eleven patients with ocular lichen planus were identified. The diagnosis was confirmed histologically for 10 patients. Nine patients were women. The average time from onset of ocular symptoms to diagnosis was 4.1 years. Eight patients had mucous membrane involvement at other sites. Disease was well controlled in eight patients. Conclusion Lichen planus should be considered in the differential diagnosis of cicatricial conjunctivitis, especially when severe lichen planus is noted at other sites.     

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.     

Dapsone in the treatment of erosive lichen planus

A patient with erosive lichen planus responded to therapy with dapsone after multiple therapeutic modalities had failed. The potential usefulness of dapsone therapy for lymphocyte-mediated inflammatory diseases such as erosive lichen planus is suggested.     

Bilateral comparison of generalized lichen planus treated with psoralens and ultraviolet A

Ten patients with generalized lichen planus were treated with oral 8-methoxypsoralen photochemotherapy (PUVA) in a bilateral comparison study. Five patients (50%) cleared completely on both sides and required no maintenance treatment after a follow-up of up to 4 years. Three other patients (30%) improved at least 50% of their previous involvement. Most of the patients experienced symptomatic improvement of the treated side by the second week of the treatment. Two patients reacted adversely and exacerbated while receiving treatment to one side of the body. While preliminary, this bilateral comparison study demonstrates that PUVA is an effective therapy for generalized, symptomatic lichen planus and suggests that maintenance therapy might not be required once complete clearance is attained. Caution should be exercised, however, since some patients might develop an exacerbation of their disease with PUVA.     

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.     

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.     

Familial lichen planus

We observed the simultaneous occurrence of generalized lichen planus in a woman and her mother. Both patients improved after therapy with topical steroid and salicylic acid ointment. Of the eighty-one cases of familial lichen planus previously reported, the vast majority (89 percent) occurred in blood relatives. The intervals of onset between familial cases were long, ranging from six weeks to thirty years (mean 73.4 months). These observations suggest that familial lichen planus may result from genetic predisposition, rather than from an infectious cause.     

Hepatitis C virus infection prevalence in lichen planus: examination of lesional and normal skin of hepatitis C virus-infected patients with lichen planus for the presence of hepatitis C virus RNA

Hepatitis C virus (HCV) is the main cause of parenterally transmitted non-A, non-B viral hepatitis. In recent years, a significant association between lichen planus and chronic HCV infection has been reported. Anti-HCV antibody status was evaluated by ELISA in 54 patients with lichen planus and 54 patients with minor dermatological disorders. PCR was used to examine HCV RNA from serum and lesional and nonlesional cutaneous biopsy samples of HCV-infected patients. Seven patients with lichen planus (12.9%) and two patients in the control group (3.7%) were anti-HCV antibody positive. Five out of seven patients with anti-HCV antibodies had demonstrable HCV RNA in lesional skin biopsies. The viral RNA was absent in three out of four patients with lichen planus whose serum samples were positive for HCV RNA and agreed to biopsy of nonlesional skin. The prevalence of HCV infection is not increased in Turkish patients with lichen planus. However our findings suggest that the virus may play a potential pathogenic role by replicating in cutaneous tissue and triggering lichen planus in genetically susceptible HCV-infected patients.