Multidisciplinary Management of Paraneoplastic Pemphigus Associated with B-Cell Lymphoma: A Case Report.

BACKGROUND

Paraneoplastic pemphigus is a rare life-threatening blistering autoimmune mucocutaneous disease associated with various neoplasms. In contrast to the skin, mucosal lesions are refractory and requires considerable healing time, heals far more slowly. A multidisciplinary approach may provide a comprehensive management and a better prognosis.

CASE SUMMARY

A sixty-one-year-old male patient presented to the ER department in June 2020 complaining of severe inflammation of the left eye along with scaly erythematous skin eruptions affecting the hands, trunk, and feet. Subungual hematoma was also observed. Clinical examination revealed extensive oral ulceration involving the dorsum and ventral surface of the tongue, buccal mucosa, labial mucosa, and hematic crusts were noticed on the lips. The patient was admitted for panophthalmitis secondary to a perforated corneal ulcer. Dermatological investigations were requested in addition to extensive baseline studies to rule out malignancies. The patient had a retroperitoneal lesion and underwent CT guided biopsy. Result was suggestive of low-grade B cell non-Hodgkin's lymphoma. He was treated by the haematology-oncology, dermatology and oral medicine teams.

CONCLUSION

Paraneoplastic pemphigus (PP) is a fatal autoimmune blistering disease associated with underlying malignancy. A multidisciplinary approach to achieve early diagnosis and better management is essential to improve the quality of life of such patients despite their poor prognosis.

CLINICAL SIGNIFICANCE

Paraneoplastic pemphigus is a rare disease associated with mucocutaneous ulcerations and various malignancies, including lymphoproliferative neoplasms. As demonstrated in the current case, multidisciplinary assessment and management have proven to be effective in managing the patient. Physicians and dentists should make their best effort to work collaboratively to manage patients and minimize patient distress and improve life quality and prognosis.