Lichen planus pemphigoides is a heterogeneous disease: a report of five cases studied by immunoelectron microscopy

Lichen planus pemphigoides (LPP) is a rare and controversial disease. It is characterized by bullae arising on lichen planus papules and on uninvolved skin, subepidermal bullae in histology, and linear deposits of IgG and C3 along the basal membrane zone on immunofluorescence of peribullous skin. Our goal was to identify the localization of the target antigen in cases of LPP. Five patients diagnosed with LPP on clinical, histological and immunofluorescence criteria were explored by immunoelectron microscopy and immunoblot. Our results show that the target antigen in LPP is not unique. The localization of the immune deposits was consistent with a diagnosis of bullous pemphigoid in two cases, of cicatricial pemphigoid in two cases and of epidermolysis bullosa acquisita in one case. Our study supports the view that LPP is a heterogeneous condition in which lichen planus may induce different subepidermal acquired bullous dermatoses.     

PUVA-induced lichen planus pemphigoides

A 72-year-old woman had suffered from parapsoriasis en plaque (large plaque type) controlled by topically applied psoralen ultraviolet A (PUVA) therapy. The parapsoriasis lesions gradually disappeared, but numerous tiny red papules with pruritus appeared over the forearms and lower legs 120 days after starting PUVA therapy. These papules developed to form violaceous plaques. Histological findings demonstrated the characteristics of lichen planus. Two months later, tense bullae developed on the plaques and on uninvolved skin of the limbs. These were subepidermal, with linear deposits of IgG and C3 along the basement membrane zone (BMZ) in immunofluorescence of peribullous skin, and immunodeposits of type IV collagen along the floor of the bullae. We therefore, diagnosed lichen planus pemphigoides (LPP). Using systemic and topical steroid therapy, the lesions rapidly resolved and there has been no recurrence. This case suggests that the combination of basal cell injuries caused by chronic inflammation and PUVA therapy could expose BMZ components to autoreactive lymphocytes and induce LPP.     

经典和色素性扁平苔藓常见皮肤镜特征的比较

目的：比较经典扁平苔藓与色素性扁平苔藓常见皮肤镜的特征。方法：选取13例经典扁平苔藓和6例色素性扁平苔藓患者共99处皮损进行皮肤镜检查并对其特征进行比较。结果：经典扁平苔藓常见的皮肤镜特征有Wickham纹,以片状模式为主的黄棕色色素结构和点状、线状及球状等血管;色素性扁平苔藓常见的皮肤镜特征有以点状、球状模式为主的蓝灰色、黄棕色色素结构和毛囊角栓。结论：皮肤镜可用于经典扁平苔癣和色素性扁平苔藓的辅助诊断。     

Bullous lichen planus developed on erythema ab igne

Lichen planus developed on erythema ab igne which was induced by repeated exposures to a stove. The clinical and histopathological features of the eruption were an admixture of typical lichen planus and erythema ab igne. Subepidermal bullae were also found on the lichen planus. Direct immunofluorescence tests revealed deposits of fibrin in the basement membrane zone and IgM in the colloid bodies. Indirect immunofluorescence studies detected no circulating antibodies against cutaneous structures in the patient's serum. This study indicates that repeated thermal exposure over a prolonged period may produce lichen planus lesions.     

A Case of Palmoplantar Lichen Planus Mimicking Secondary Syphilis

Lichen planus is a chronic inflammatory disease that affects skin, mucous membranes, hairs, and nails. According to the site of involvement, lichen planus can be subdivided into palmoplantar, mucosal, nail, or scalp types. Palmoplantar lichen planus is a rare subtype of lichen planus which may create difficulty in diagnosis, as the lesion is limited to the palms and soles. We report a case of a 37-year-old male with lichen planus who presented with multiple erythematous, scaly, hyperkeratotic papules on his palms and soles mimicking secondary syphilis.     

呈网状红斑改变的扁平苔藓

报告1例呈网状红斑改变的扁平苔藓。患者女,34岁。全身泛发网状红斑22年,伴瘙痒,有口腔黏膜及甲受累。皮肤科检查见全身泛发网状红斑,部分红斑消退后局部有轻度萎缩,散在水疱,尼氏征阴性。双手多个指甲甲板萎缩、吸收。上腭见片状糜烂面。组织病理检查示表皮变薄,基底细胞广泛液化变性,真皮浅层淋巴细胞呈带状浸润。诊断：扁平苔藓。给予阿维A40mg/d口服3个月后皮损明显变平,颜色变暗。     

Lichen Planus Pigmentosus Presenting in Zosteriform Pattern

Lichen planus pigmentosus (LPP) has thus far been described as a condition of unknown etiology which clinically differs from the classical lichen planus (LP) by exhibiting dark brown macules and/or papules mostly in exposed areas and flexural folds and a longer clinical course without pruritus or scalp, nail or mucosal involvement. Histopathologically, LPP shows the typical changes seen in LP, but with thinning of epidermis. We report a case of LPP that developed in a unilateral, zosteriform pattern on the left flank of a 49-year-old man. This case seems to lie in the middle of the spectrum between classical LP and ashy dermatosis, and, to the best of our knowledge, is the first report of LPP presenting in the zosteriform pattern.     

Lichen planus pigmentosus–inversus

We examined seven patients with lichen planus pigmentosus (LPP) clinically and microscopically. Clinically, all patients had a striking predominance of lesions in an intertriginous location, with most of them in the axillae. Microscopically, two biopsies were of significance. Except for the regressive lichen planus, which is usual in LPP, the active inflammatory phase was also present. In these biopsies the very intensive hydropic degeneration of basal keratinocytes was combined with the absence of compensatory increased proliferation of keratinocytes, i.e. without acanthosis. The short duration of this process probably led to the quick transformation into a long noninflammatory regressive phase with incontinence of the pigment. These specific morphogenetic dynamics are possibly why most of the morphs of LPP present as brown, non-pruritic, small inflammatory macules. Because of the highly characteristic inverse location of the lesions in our patients we propose the designation LPP-inversus for this variant of the disease.     

Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon

An 18-year-old girl presented with multiple itchy hyperpigmented papules and plaques, along with tense blisters over the lower limbs and buttocks for last 3 months. These papules, plaques, and bullae were mostly localized to preexisting scars. The histopathological findings from papule and bulla were consistent with lichen planus (LP) and bullous pemphigoid, respectively. Direct immunofluorescence (DIF) of perilesional skin around bulla showed linear deposition of IgG and C3. Considering clinical, histopathological and DIF findings, diagnosis of LP pemphigoides (LPP) was made. The preferential localization of LPP lesions over preexisting scars was a very interesting finding in our case an extremely rare instance of the isotopic phenomenon.     

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.     

THERAPEUTIC HOTLINE: Effectiveness of the association of cetirizine and topical steroids in lichen planus pilaris – an open‐label clinical trial

Lichen planus is considered a T cell‐mediated immunological disease. Even mast cells may contribute to the pathogenesis of the disease. Keratinocytes of the basal layer of the skin and/or the hair follicle may represent the “target/victim” of an immune aggression, determining the destruction of the hair follicle and thus scarring alopecia. Therefore, there is a compelling urgency for effective treatment of this potentially disfigurating dermatosis. Our data provide a further therapeutic opportunity: the use of an antihistaminic drug – cetirizine (CTZ) – in an “anti‐inflammatory” regimen. We propose the use of CTZ at the dosage of 30 mg/daily. Twenty‐one patients affected by lichen planus pilaris (LPP) of the scalp have been treated. Topical application of steroids has been coadministrated in all cases during the therapy. Clinical effects, in the sense of stabilization with cessation of the inflammation (erythema, follicular hyperkeratosis, loss of anagen hair), were achieved in all patients but three. One patient developed cardiac arrhythmia after 3 months of successful treatment and dropped out. Our cases indicate that a combined therapy of topical steroid with CTZ can be a safe and effective choice even in severe cases of lichen planus pilaris, so often refractory to the therapy.     

Pemphigus vulgaris coexisting with generalized lichen planus

A case of coexistence of pemphigus vulgaris and generalized lichen planus is presented. The patient had clinical, histological and immunopathological findings of pemphigus vulgaris in the mouth, lichen planus on the scalp, and glabrous skin over the trunk and the extremities. These facts may suggest that a common immunologic mechanism may cause either disease, depending on the constitutional predisposition of the patient.     

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.     

Plantar lichen planus masquerading eumycetoma of dark grains

Lichen planus is a common inflammatory disease but its perforating variety is not so common and it has been described in small number of text and articles. Here we reported a case of plantar lichen planus where there was a history of discharge of dark grains from the sole of foot and diagnosing the disease as eumycetoma of dark grains repeated antifungal therapy could not resolve the lesions and histopathologically it showed the classical pictures of lichen planus. Collaborating the clinical and histological features we have diagnosed the case as perforating lichen planus but Verhoeff-Van Gieson stain could not elucidate the perforating channel which ot difficult to delineate and often missed. So, we have put the diagnosis of plantar lichen planus and treated with intramuscular triamcinolone and the lesions resolved.     

Erosive mucosal lichen planus: response to topical treatment with tacrolimus

Erosive mucosal lichen planus is a painful and disabling inflammatory skin disease that is highly resistant to topical treatment. We report on six patients with severe recalcitrant erosive mucosal lichen planus who benefited from topical application of tacrolimus ointment. After 4 weeks of treatment, complete resolution was observed in three cases, and substantial improvement was achieved in the other three patients. In these cases, prolonged treatment resulted either in further improvement or in complete healing. All patients reported rapid relief from pain and burning. No severe side-effects were observed.     

Mucosal vulval lichen planus: outcome, clinical and laboratory features

BACKGROUND: Mucosal lichen planus of the vulva is a rare but increasingly recognized condition. It has potentially severe complications such as fusion of the labia and vagina; the risk of developing squamous cell carcinoma (SCC) may be increased. An association between hepatitis B and C infection and skin or oral lichen planus appears to exist in certain geographical areas. OBJECTIVE: To investigate the course of mucosal vulval lichen planus, its response to treatment and associated laboratory features. SUBJECTS AND METHODS: Forty-four women with mucosal vulval lichen planus were studied between 1997 and 2000 and laboratory data were collected. RESULTS: Thirty of 44 patients had additional oral lesions, only nine had cutaneous findings compatible with lichen planus. We did not find an association with antibodies to hepatitis B or C virus in this British study population. All women were treated with potent to very potent topical corticosteroids; however, in the majority of patients symptoms persisted. In seven (16%) patients vulval lichen planus was in remission after a disease duration between 2 and 18 years (mean 10.6 years). One patient developed a vulval SCC. CONCLUSIONS: Screening for hepatitis B and C in women with mucosal vulval lichen planus in the UK seems unnecessary. We recommend long-term follow-up, and that all non-healing ulcerative and papular lesions should be biopsied.     

Oral lichen planus: case series and experience in a tertiary dermatology service in Brazil

BackgroundLichen planus is an inflammatory disease that can affect both the skin and mucous membranes, including the oral mucosa. There is very little original Brazilian dermatology literature about oral lichen planus.ObjectiveTo describe the clinical, pathological, and treatment data of 201 patients diagnosed with oral lichen planus followed at the Stomatology Outpatient Clinic of Hospital das Clínicas, Universidade de São Paulo, from 2003 to 2021.MethodThe patients demographic profile, the morpho-topographic features of the lesions, the treatment employed, and the possible presence of squamous cell carcinoma were analyzed.ResultsThe disease was more common in women over 50 years of age, tending to be chronic, with a large number of cases showing cicatricial sequelae in the mucosa. Topical treatment with potent corticosteroids was shown to be effective in the vast majority of cases. Squamous cell carcinoma in oral lichen planus cicatricial sequelae was observed in eight cases.Study limitationsRetrospective study of medical records, with gaps regarding the filling out of data; unequal observation time among the studied cases.ConclusionsThis is the largest Brazilian dermatology series on oral lichen planus. The response to topical corticoid therapy was excellent in the vast majority of cases. The high prevalence of atrophic lesions, demonstrating the chronicity and tissue destruction potential of this disease, may explain the large number of cases of squamous cell carcinoma.     

Ulcerative lichen planus associated with Sjögren's syndrome

Ulcerative lichen planus is a rare variant of lichen planus that is characterized by ulcerations of the feet and toes that are accompanied by toenail loss. However, the nail, oral mucosa, genital mucosa and the scalp are also sometimes affected by ulcerative lichen planus. Several authors have drawn attention to the association of ulcerative lichen planus and autoimmune diseases. We report a patient who had ulcerative lichen planus, with ulcerative erythema on the soles and palms, nail dystrophy and oral lesions, as well as Sjögren's syndrome; she was successfully treated with etretinate.     

Lichen planus pigmentosus inversus in children: Case report and updated review of the literature

Lichen planus pigmentosus inversus (LPPI) is a rare variant of lichen planus characterized by slate grey to dark black‐brown macules, papules, or patches occurring in the skin folds. We present a case of LPPI in an 11‐year‐old girl, the second‐youngest case and only the third pediatric case. This article also reviews the differential diagnosis and treatment of LPPI.     

Ultraviolet A-1 as a treatment for ulcerative lichen planus of the feet

Ulcerative lichen planus of the feet is a rare variant of lichen planus characterized by the constellation of a chronic bullous and ulcerative eruption limited to the feet, atrophy of involved skin and nails, and cicatricial alopecia. Only split-thickness skin grafting has here to been documented to be effective. We here report a case with ulcerative lichen planus of the feet successfully treated with medium-dose ultraviolet A-1.