Paraneoplastic Pemphigus and Myasthenia Gravis associated with Unicentric Castleman Disease: A Case Report
Narachai Julanon1, Charoen Choonhakarn1, Piti Ungarereevittaya2, Suteeraporn Chaowattanapanit1
Affiliation : 1 Division of Dermatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand 2 Department of Pathology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
Background: Paraneoplastic pemphigus is a rare autoimmune blistering skin disease presenting with intractable erosive stomatitis and polymorphic cutaneous lesions including pemphigus-like lesions, bullous pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like manifestations. Associated neoplasms are non-Hodgkin lymphoma, chronic lymphocytic leukemia, and Castleman disease. The mortality rate of this disease was high.
Case Report: The authors reported a case of a 24-year-old man who had paraneoplastic pemphigus and myasthenia gravis associated with unicentric Castleman disease. He presented with stomatitis and ulcerated lichenoid lesions on his hands and feet, along with paronychial involvement and nail dystrophy for many months. Histopathological findings from the skin lesions showed vacuolar interface dermatitis. Direct immunofluorescence demonstrated deposition of IgG and C3 at the dermo-epidermal junction. Indirect immunofluorescence using rat bladder epithelium revealed circulating IgG antibodies in the intercellular space. Additionally, he had ptosis and proximal muscle weakness due to myasthenia gravis and left upper quadrant abdominal mass, with histological confirmation of unicentric Castleman disease, hyaline vascular type. The diagnosis of paraneoplastic pemphigus and myasthenia gravis associated with unicentric Castleman disease was made. He had initially been treated with prednisolone and mycophenolate mofetil. Tumor removal was later performed. Mucocutaneous lesions and weakness gradually improved within three months after the operation. Currently, the disease has been well controlled with prednisolone.
Conclusion: Paraneoplastic pemphigus can present as intractable stomatitis and lichen planus-like lesions. High suspicion can lead to proper investigations to confirm the diagnosis and subsequently seek an associated neoplasm. The treatment includes treatment of associated neoplasm and a combination of systemic corticosteroids with immunosuppressants, including mycophenolate mofetil for this patient.
Received 6 February 2024 | Revised 21 February 2024| Accepted 30 February 2024
DOI: 10.35755/jmedassocthai.2024.S01.S152-S156
Keywords : Paraneoplastic pemphigus; Myasthenia gravis; Castleman disease; Case report
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