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Case Reports
. 2025 Feb 22;17(2):e79459.
doi: 10.7759/cureus.79459. eCollection 2025 Feb.

Paraneoplastic Pemphigus in a Patient With Pheochromocytoma: A Report of a Rare Case

Zhong-Zhou Huang  1 , Ming-Jie He  1 , Ping Huang  2 , Min-Qing Luo  1 , Dan Hong  1 , Sha Lu  1 , Qing Guo  1 , Liangchun Wang  1 , Hui Xiong  1
Affiliations
Case Reports

Paraneoplastic Pemphigus in a Patient With Pheochromocytoma: A Report of a Rare Case

Zhong-Zhou Huang et al. Cureus. .

Abstract

This report details a rare case of paraneoplastic pemphigus (PNP) associated with pheochromocytoma. The patient presented with prominent dermatological manifestations, including erythema, vesicles, and erosions. During hospitalization, diagnostic imaging revealed a retroperitoneal mass, which was subsequently surgically removed. A comprehensive diagnostic workup, including CT, MRI, histopathological analysis, and direct immunofluorescence examination, was conducted. Postoperative management combined with pharmacological intervention led to significant clinical improvement. This case highlights the critical importance of considering PNP in the differential diagnosis of pheochromocytoma, particularly in patients presenting with complex autoimmune manifestations. The findings underscore the necessity for early diagnosis and prompt therapeutic intervention in such cases. Additionally, this report emphasizes the need for further investigation into the clinical spectrum, genetic associations, and underlying mechanisms of this rare disease association to enhance diagnostic accuracy and therapeutic outcomes.

Keywords: autoantibody; autoantigen; autoimmune diseases; desmoglein; methotrexate; methylprednisolone; paraneoplatic pemphigus; pemphigus vulgaris; pheochromocytoma; rituximab.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Physical examination revealing erosive blisters and scabs (arrows).
A) face; B) neck; C) chest; D) back Written informed consent has been provided by the patient to have the case details and any accompanying images published.
Figure 2
Figure 2. Histopathological examination of the abdominal skin biopsy (arrows).
A) Histopathological examination, showing parakeratosis and crust formation, with scattered necrotic keratinocytes, liquefaction degeneration of basal cells, and infiltration of lymphocytes in the superficial dermis (H&E staining, x200); B) Direct immunofluorescence showing intercellular deposition with IgG positive reactivity.
Figure 3
Figure 3. Clinical detection in plasma.
A) Both titers of anti-Dsg1 and anti-Dsg3 in plasma showing a gradual decrease in both antibodies since the clinical management, including the laparoscopic resection on April 18, 2024. B) Both levels of methoxyephedrine and methoxy-norepinephrine in plasma revealing a decrease, especially in methoxyephedrine since the laparoscopic resection. Dsg: desmoglein
Figure 4
Figure 4. Retroperitoneal mass and examination (arrows).
A) Computed tomography (CT), showing the mass; B) Magnetic resonance imaging (MRI), showing the solitary mass; C) Retroperitoneal mass, approximately 6 x 5 x 2.5 cm3; D) Histopathological examination (HE staining, x200), showing atypical cells with partial cellular atypia; E) Histopathological examination (HE staining, x40).
See this image and copyright information in PMC

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