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Indian J Dermatol
Indian J Dermatol
IJD
Indian J Dermatol
Indian Journal of Dermatology
0019-5154
1998-3611
Wolters Kluwer - Medknow India

IJD-69-346
10.4103/ijd.ijd_169_24
Correspondences
Can Lichen Planus be a Forerunner of Paraneoplastic Pemphigus?
Padiyath Mohamed Twaseem
Criton Sebastian
Abraham Usha Mary 1
From the Department of Dermatology, Amala Institute of Medical Sciences, Thrissur, Kerala, India E-mail: twaseem16@gmail.com
1 Department of Pathology, Amala Institute of Medical Sciences, Thrissur, Kerala, India
Jul-Aug 2024
19 8 2024
69 4 346348
2 2024
3 2024
Copyright: © 2024 Indian Journal of Dermatology
2024
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pmcDear Editor,

A 30-year-old female was referred to our outpatient department with oral erosions for the past 6 months. On examination, there were violaceous plaque and erosions in the buccal mucosa and tongue [Figure 1]. Biopsy was done and histopathology showed epidermal acanthosis and dermal lymphocytic infiltrate with plasma cell and civette bodies [Figure 2]. Thus, the patient was diagnosed with oral lichen planus and was started on prednisolone of 30 mg.

Figure 1 Violaceous plaque and oral erosions in buccal mucosa and tongue

Figure 2 Epidermal acanthosis and dermal lymphocytic infiltrate with plasma cell and civette bodies (H and E staining ,10x magnification)

After 3 months of treatment, the patient presented with an exacerbation [Figure 3], along with progressive dyspnoea and cough. Owing to inadequate clinical response and pulmonary symptoms, the possibility of lichenoid eruption of paraneoplastic pemphigus (PNP) was considered. Thus, biopsy with direct immunofluorescence (DIF) was repeated; indirect immunofluorescence (IIF) and antidesmoglein 1,3 were sent. Histopathology showed dermal infiltrate of predominantly plasma cell, neutrophil and lymphocyte suggestive of chronic inflammation [Figure 4]. DIF showed intercellular zone immunoglobulin G (IgG) 2+. IIF showed IgG intercellular zone+ (1:100). Rat bladder could not be done due to the unavailability. This led us to revise the diagnosis as pemphigus vulgaris.

Figure 3 Exacerbation of oral erosions

Figure 4 Dermal infiltrate of predominantly plasma cell, neutrophil and lymphocyte (H and E staining ,10x magnification)

We had high alertness to pulmonary symptoms considering the possibility of bronchiolitis obliterans, but evaluation pointed out obstructive airway disease. Owing to high clinical suspicion of underlying malignancy, computed tomography (CT) chest and abdominal ultrasound were done but were found to be normal.

The oral erosions persisted despite systemic treatment for 10 months. Thus, tumour markers were sent, despite the negative evidence for PNP. Cancer antigen (CA) 19.9 was found to be elevated. Furthermore, repeat whole-body CT showed soft tissue lesions in the root of mesentery and retroperitoneal lymph nodes [Figure 5]. Histopathology showed Grade 1 follicular lymphoma. Thus, the diagnosis was revised after 11 months as PNP with non-Hodgkin lymphoma. The oral lesions improved after chemotherapy [Figure 6].

Figure 5 Soft tissue lesion in the root of mesentery encasing mesenteric vessels and prominent multiple retroperitoneal lymph node (CT scan)

Figure 6 Post treatment

In 1990, Anhalt described PNP as a blistering disease characterised by acantholytic polymorphic mucocutaneous eruption and autoantibodies against adhesion proteins.[1] In the case presented earlier, oral lesions were diagnosed as PNP after 11 months of gruelling search. Thus, retrospectively the lichen planus-like presentation could have been a lichenoid variant of PNP. But clinically and histopathologically there was no evidence. A retrospective immunohistochemistry of lichen planus lesion showed a polyclonal T cell population of CD8 T cells, thus reconfirming diagnosis. The contrasting histopathology just over a period of 3 months led to the question “Could lichen planus-like paraneoplastic manifestation transform into pemphigus?”

Our literature search found that Bowen et al.[2] in a case series of six patients with PNP observed that lichen planus was present clinically and histopathologically in five patients. It not only co-existed, rather it even preceded PNP by years and they hypothesised “epitope spreading” as an explanation. Thus, the above case scenario can be explained as “Cell mediated immunity by cytotoxic T cell triggered by malignancy against the basement membrane further exposes the antigens, and eventually potentiates the development of B cell immunity through recognition by autoreactive T cells.”[2] A case series of four patients by Cumins et al.[3] further supports this and thus the diagnostic criteria was revised. The case presented earlier could be supporting evidence for this literature.

The practising physicians may have to consider paraneoplasia in case of recalcitrant oral lichenoid lesion even in the absence of detectable antibodies. Therefore their treatment should target the prominent T cell along with B cells.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
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1 Anhalt GJ Kim SC Stanley JR Korman NJ Jabs DA Kory M Paraneoplastic pemphigus-An autoimmune mucocutaneous disease associated with neoplasia N Engl J Med 1990 323 1729 35 2247105
2 Bowen GM Peters NT Fivenson DP Su LD Nousari HC Anhalt GJ Lichenoid dermatitis in paraneoplastic pemphigus: A pathogenic trigger of epitope spreading? Arch Dermatol 2000 136 652 6 10815859
3 Cummins DL Mimouni D Tzu J Owens N Anhalt GJ Meyerle JH Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies J Am Acad Dermatol 2007 56 153 9 17097371
