• Ahmad Bakhtiar Md Radzi and Sazzli Shahlan Kasim.
• Department of Cardiology, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh Campus, Sungai Buloh, Selangor, Malaysia.
• Medicine (Baltimore). 2023 Jan 27; 102 (4): e32822e32822.

RationaleWe report a rare case of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma.Patients ConcernsThe patient presented with 5 months' history of generalized skin itchiness, night sweat and loss of weight. The skin manifestations started over the foot and hand area. However, he started to developed tense blisters over the face, trunk and limbs 3 days prior to this admission.DiagnosesThe skin biopsy report showed subepidermal bullae, in which the immunofluorescence findings in keeping with bullous pemphigoid. The peripheral blood immunophenotyping was suggestive of mantle cell lymphoma. Hence, a diagnosis of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma was made.InterventionsThe patient was initiated with a cytoreduction chemotherapy.OutcomesUnfortunately, patient's condition deteriorated further due to neutropenic sepsis and he succumbed after 2 weeks of intensive care.LessonsBullous pemphigoid associated with mantle cell lymphoma are very rare. The presentation of bullous pemphigoid led to the detection of mantle cell lymphoma. Early diagnosis and appropriate treatment is crucial in managing this aggressive type of the disease. Both, bullous pemphigoid and mantle cell lymphoma had a parallel clinical course which suggests a paraneoplastic phenomenon in this reported case.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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