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130:Exacerbation of Erosive Lichen Planus or Paraneoplastic Tumour?[AAOM2021]
EP36060
Poster Title: 130:Exacerbation of Erosive Lichen Planus or Paraneoplastic Tumour?[AAOM2021]
Submitted on 29 Mar 2021
Author(s): Safa Mohamed
Affiliations: Bristol Royal Infirmary
This poster was presented at 2021 American Academy of Oral Medicine Virtual Conference
Poster Views: 392
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Poster Information
Abstract: A female in her twenties presented to the Emergency Department with severe widespread painful oral ulceration and atrophic lesions resulting in reduced oral intake. She reported months of malaise, difficulty managing due to her oral pain along with a 2-week history of a productive, chesty cough with shortness of breath. Other complaints included: oral swelling, difficulty speaking, difficulty eating, bleeding gums, sore throat and sleep disturbance. She denied nausea, vomiting, fever, night sweats or weight loss. She was previously diagnosed with erosive lichen planus which was refractory to treatment and under regular review by the oral medicine department. She was otherwise fit and well, a non-smoker and rarely consumed alcohol.

Haematological assessment revealed raised C-reactive protein at 17mg/L. The ECG corresponded to the vital signs and demonstrated sinus tachycardia. Covid-19 test was negative. However, chest radiograph revealed a well circumscribed lesion in the right base of lung. Computed tomography thorax with contrast chest demonstrated a 5.5 cm soft tissue mass in the right lower lobe of the lung. Furthermore, there was an enlarged lymph node located on the right hilum.

An upper left lip perilesional biopsy revealed histological features typical of lichenoid type inflammatory infiltration and basal cell damage. Furthermore, intraepithelial clefting was visible above the level of the basement membrane. Direct immunofluorescence revealed intercellular deposits of C3 along with IgG deposits within the epidermis. This histopathology supported a diagnosis of pemphigus. A right lung bilobectomy involving the middle and lower lobes, and lymphadenectomy. The histopathology of this lobectomy revealed features representative of unicentric hyaline-vascular type of Castleman’s Disease. The final diagnosis was paraneoplastic pemphigus with underlying unicystic Castleman’s disease

Paraneoplastic pemphigus is a fatal autoimmune blistering disorder which can be challenging to diagnose due to its polymorphic appearance and rarity. This case highlights the need to exclude paraneoplastic pemphigus in patients with oral lichen planus that is refractory to treatment. Referral to a local oral medicine department for further specialist investigation is warranted in cases where conventional symptomatic treatment is unsuccessful, or if there is a rapid and significant deterioration in the clinical picture.
Summary: Paraneoplastic pemphigus is a fatal autoimmune blistering disorder which can be challenging to diagnose due to its rarity and polymorphic appearance. This case highlights the need to exclude paraneoplastic pemphigus in patients with oral lichen planus that is refractory to treatment, or if there is a rapid and significant deterioration in the clinical picture.

Ask the author questions about this poster:safaomar1234@hotmail.com
References: 1. Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunologic Research. 2018;66(2):255-270.
2. Anhalt G, Kim S, Stanley J, Korman N, Jabs D, Kory M et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous
disease associated with neoplasia. New England Journal of Medicine. 1990;323(25):1729-1735.
3. Soumerai J, Sohani A, Abramson J. Diagnosis and Management of Castleman Disease. Cancer Control. 2014;21(4):266-278
4. Castleman B, Iverson L, Menendez V. Localized mediastinal lymph-node hyperplasia resembling thymoma. Cancer.
1956;9(4):822-830..
5. Dispenzieri A, Fajgenbaum D. Overview of Castleman disease. Blood. 2020;135(16):1353-1364.
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