Bullous lichen planus - a review.

Lichen planus

Two patients with lichen planus pemphigoides and two with bullous lichen planus were compared. Lichen planus pemphigoides was clinically distinguished by a more generalized lichen planus, more extensive blistering, the need for systemic corticosteroids and by a longer course. The blister of bullous lichen planus was a subepidermal bulla showing degeneration of the epidermal basal layer and other features of lichen planus, whereas in lichen planus pemphigoides the bulla was similar to that of bullous pemphigoid albeit with rather more neutrophils than are usually seen. Direct immunofluorescence was positive in lichen planus pemphigoides and negative in bullous lichen planus. Lichen planus pemphigoides and bullous lichen planus are separate entities: the former is an auto-immune disease precipitated by lichen planus and not related to bullous pemphigoid, the latter is probably not auto-immune but represents the extreme consequence of the lymphoid infiltrate at the dermo-epidermal junction.

Bullous lichen planus is a rare variant of lichen planus. It is characterized by vesicles or bullae, which usually develop in the context of pre-existing LP lesions. The clinical features of bullous lichen planus include typical lichen planus lesions, accompanied by the formation of bullae on the affected or perilesional skin. The clinical features of bullous lichen planus include typical lichen planus lesions, accompanied by the formation of bullae on the affected or perilesional skin.

Lichen planus pemphigoides describe a rare subset of patients who usually have typical lichen planus then develop blistering on their lichen planus lesions and in normal skin. Less commonly the blistering antedates the lichen planus. They clinically appear to be a combination of lichen planus and bullous pemphigoid. Oral disease may occur and resemble either lichen planus or bullous pemphigoid. Lichen planus pemphigoides has been triggered by medication & PUVA. Pruritus may be severe and lesions may evolve to resemble pemphigoid nodularis. Histopathologically lichen planus lesions show lichen planus and bullous lesion shows the features of bullous pemphigoid. DIF is positive in a linear pattern with IgG and C3 along the basement membrane zone, at the roof of saline split skin. The antigen targeted by the autoantibody in Lichen planus pemphigoides is located in the same region as the bullous pemphigoid antigen (at the basal hemidesmosomes). Lichen planus pemphigoides tends to follow a benign and chronic course, even when compared to bullous pemphigoid. We diagnosed a case of Lichen planus pemphigoides on the basis of history, clinical examination, histopathology & DIF. The patient was treated with systemic & topical steroid, Dapsone. After 2 month of treatment steroid was withdrawn, but Dapsone continue with no relapse.To our knowledge this is the first diagnosed and treated case in this hospital. DOI: http://dx.doi.org/10.3329/jssmc.v4i1.12002 J Shaheed Suhrawardy Med Coll, 2012;4(1):35-37

Lichen planus pemphigoides: another paraneoplastic bullous disease?

Lichen planus pemphigoides (LPP) and bullous lichen planus (BLP) are rare dermatoses, which are characterised by blisters and lichenoid lesions. Their clinical presentation is heterogenous, displaying overlapping features or mimicking other dermatological diseases. Therefore, diagnosis can often be challenging, requiring a thorough dermatological examination along with distinctive histological and immunopathological characteristics. Lichenoid degeneration of the basal epidermis exposes various antigens of the dermal-epidermal junction in LPP, resulting in the breakdown of immune tolerance, hence, the production of autoantibodies against type XVII collagen. Conversely, no pathogenic autoantibodies are detected in BLP. However, some cases of mucosal lichen planus might display immunopathological features suggestive of autoimmune blistering diseases. Therefore, a better understanding of the pathophysiology of these two distinct dermatoses is imperative. The aim of this review was to provide a summary of the current knowledge on the clinical hallmarks, diagnosis and available therapeutic options in LPP and BLP.

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 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 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.

Autoantibodies in Lichen Planus Pemphigoides React with a Novel Epitope within the C-Terminal NC16A Domain of BP180

Lichen planus is a chronic inflammatory disorder that may affect the skin, nails, and/or oral mucosa. Bullous lichen planus is a rare variant of lichen planus, which is even less common in the nails. We present a case of nail bullous lichen planus, in a 48-year-old male presenting with a 10-month history of onychodystrophy of all ten fingernails. A longitudinal excision of the left thumbnail was performed, with histopathology consistent with lichen planus with focal transition to bullous lichen planus. He was treated with intralesional triamcinolone injections to the fingernails monthly, with improvements noted after three treatments. Our patient’s nail bullous lichen planus manifested with longitudinal ridging, white-yellow discoloration, onycholysis, subungual hyperkeratosis, and v-shaped nicking. Histopathological findings included classical lichen planus changes, as well as formation of subepidermal bullae, colloid bodies, and extensive inflammatory infiltrate. Increased awareness and high index of suspicion for this condition are necessary, given the often late diagnosis reported in previously published cases.

Lichen planus pemphigoides (LPP) is an autoimmune disease characterised by evolution of subepidermal blisters on normal and lichen planus affected skin. We describe a case of LPP in a 54-year-old Chinese woman. The patient presented with psoriasiform plaques and was diagnosed with guttate psoriasis. Narrowband ultraviolet B (NBUVB) therapy was commenced, and she experienced a generalised eruption of violaceous papules, bullae over the lower limbs, and Wickham's striae over the buccal mucosa. Histology from a plaque revealed interface dermatitis, while a specimen from a blister showed subepidermal bulla. Direct immunofluorescence showed linear deposition of IgG and C3 along the basement membrane. A diagnosis of LPP was made on clinicopathological grounds. This is the first case report of NBUVB alone in unmasking LPP. In this case report, we describe the pathological mechanism of NBUVB in the development of LPP and key features distinguishing LPP from bullous lupus erythematosus, bullous lichen planus, bullous pemphigoid, and psoriasis.

Background: Oral lupus erythematosus (OLE) and oral lichen planus (OLP) are among the common causes of oral lichenoid lesions (OLLs). The differential diagnosis among causes of OLLs, particularly between OLE and OLP, is challenging as they have significant clinical and histopathological overlap. Objectives: To compare and summarize the clinical, histopathological, and direct immunofluorescence (DIF) findings between OLE, OLP, and other OLLs and to explore the diagnostic value of CD123 immunohistochemistry. Methods: A retrospective study on patients with OLE, OLP, and other OLLs was performed between January 2014 and December 2019. The baseline characteristics, the clinical, histopathological, and DIF features, as well as CD123 immunohistochemistry for plasmacytoid dendritic cells (PDCs) were statistically analyzed and compared between groups. Results: Of 70 patients, 12 had OLE, 39 had OLP, and 19 had other OLLs. Oral erosions/ulcers were the most common findings in all three groups. Red macules, telangiectases, and discoid plaques were more common in OLE patients, while OLP cases were typified by reticulated patches (p < 0.05). Additionally, white patches were found more often in other OLLs than in both OLE and OLP (p = 0.002). Histologically, mucosal atrophy, basal vacuolization, and perivascular infiltrate were observed in OLE, whereas OLP specimens possessed mucosal hyperplasia, hypergranulosis, and compact orthokeratosis (p < 0.05). Mucosal spongiosis was a histologic feature that favored other OLLs over OLE and OLP (p < 0.001). Data on DIF were nonspecific for all three conditions. For immunohistochemical staining, the median number of total CD123+ PDCs was observed to be higher in OLE than OLP in the mucosal-submucosal junction (MSJ) (p = 0.021), the superficial perivascular area (p = 0.026), and the superficial and deep perivascular areas (p = 0.001). Likewise, PDCs in clusters ≥2+ were seen in significantly higher numbers on OLE than OLP along the MSJ (p = 0.002), the superficial perivascular area (p < 0.001), as well as the superficial and deep perivascular areas (p = 0.011). CD123+ PDCs were found to be significantly more numerous in both OLE and OLP than other OLLs in all of the abovementioned areas (all p < 0.05). Conclusion: While there are some differences in the clinicopathological features between OLE, OLP, as well as other OLLs, a significant overlap remains. The quantity and distribution pattern of CD123 immunohistochemical staining has a diagnostic implication in differentiating OLE from OLP and other OLLs.

Objective: Oral bullous lichen planus (BLP) is an uncommon form of lichen planus that affects just 1% of the oral mucosa. It is seen in oral mucous, palate, buccal mucous, and occasionally in the tongue. The purpose of this case report is the importance of including lichen planus in the differential diagnosis of leukoplakia-like lesions.Case: The presentation included lateral parts of the tongue, sublingual, and hyperkeratotic areas on the cheek, as well as asymptomatic bullous lichen planus phenomena, clinical and histological characteristics that have been present for 5 years. Systemic corticosteroid was given to the patient who had no skin symptoms. Conclusion: Burning sensation, the BLP's common clinical symptom, which is infrequently observed in the oral mucosa, may not be visible in all patients. In addition, BLP can be confused with leukoplakia when observed in plaque form. Histopathological examination is mandatory for definitive diagnosis in terms of malignant potential

Background:Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disease presenting with lichenoid papules and plaques and tense blisters. There is a paucity of literature on LPP globally.Objective:To report the clinico-demographic profile, histopathology, immunological features, and associated comorbidities in LPP patients.Patients and Methods:This was a retrospective study, where past records of all LPP patients diagnosed and treated between November 2013 and October 2022 were included. Patients having a compatible clinical presentation with histopathological and immunological (direct immunofluorescence) evidence of LPP were included.Results:There were 12 LPP patients, with a female-to-male ratio of 2:1. The mean age at diagnosis was 49.6 years and the mean duration of illness before presentation was 3.1 years. Clinical presentation included tense blisters and lichenoid lesions. Oral mucosal involvement was seen in six (50%) patients. Comorbidities were present in three patients. Histopathology showed a subepidermal split in 10 (83.3%), basal cell damage and pigment incontinence in four (33.3%), hypergranulosis and apoptotic keratinocytes in two (16.7%), and lichenoid infiltrate in papillary dermis in one (8.3%) patient. Perilesional direct immunofluorescence (DIF) revealed linear deposits of immunoglobulin G (IgG) and complement component 3 (C3) at the dermo-epidermal junction. The salt-split indirect immunofluorescence done in three patients showed roof binding. Enzyme-linked immunosorbent assay (ELISA) done in three patients showed antibodies against BP180. The majority of patients (83.3%) were treated with oral prednisolone, either alone (16.7%) or in combination (83.3%) with adjuvants.Limitations:Retrospective design and small sample size are the limitations.Conclusion:LPP is a rare subepidermal blistering disorder seen more commonly in adult females. DIF, ELISA, and salt-split indirect immunofluorescence are helpful tools in confirming the diagnosis of LPP and differentiating from bullous lichen planus. Oral corticosteroids comprised the mainstay of therapy. Azathioprine or dapsone were commonly used adjuvants.

The immunofluorescence studies in 12 cases of bullous lichen planus (LP) are reviewed. The clinical and immunological findings suggest that there are two different forms of bullous LP: firstly, LP vesiculosus which is an acute form of classical LP, and, secondly, LP pemphigoides which is merely the association of LP and bullous pemphigoid.