Molla Yohannis Derbew, Answar Isak Omer, Worku Biruk Mulat, Tadesse Amanuel Kassa, Tefera Elias Manaye, Alemu Bewketu Abebe, Legese Gebrehiwot Lema, Abera Samuel Addisu
Departments of Surgery.
Internal Medicine.
Ann Med Surg (Lond). 2024 Mar 6;86(4):2256-2261. doi: 10.1097/MS9.0000000000001920. eCollection 2024 Apr.
Paraneoplastic pemphigus (PNP) is an uncommon autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphous skin eruptions, and the presence of underlying neoplasms. Unique histopathological features include suprabasal acantholysis and clefts with scattered necrotic keratinocytes.
A 27-year-old female patient presented with a 3-month history of a painless swelling, approximately the size of a pea, on the left lateral aspect of her neck and axillary area. This swelling progressively increased in size and number. Additionally, she had reddish, itchy, raised skin lesions over her elbows bilaterally, which gradually spread to involve most of her body, including her lips, tongue, and buccal mucosa. These skin lesions were associated with difficulty swallowing both liquid and solid foods. A diagnostic test, including a biopsy, confirmed the diagnosis of PNP. Subsequently, the patient was managed with chemotherapy and other supportive measures, leading to improvement and eventual discharge.
PNP is a rare blistering disorder associated with neoplasms, often presenting diagnostic and treatment challenges. Patients with PNP may develop a diverse range of lesions. It is crucial to promptly recognize and manage the underlying malignancy for improved patient outcomes.
This case highlights the rare association between T-cell lymphoma and PNP. Clinicians should also remain vigilant for the possibility of PNP in lymphomas that are not of B-cell lineage.
副肿瘤性天疱疮(PNP)是一种罕见的自身免疫性黏膜皮肤疾病,其特征为严重的口腔炎、多形性皮肤疹以及潜在肿瘤的存在。独特的组织病理学特征包括基底层上棘层松解和裂隙,伴有散在的坏死角质形成细胞。
一名27岁女性患者,左侧颈部和腋窝区域出现无痛性肿胀,约豌豆大小,病史3个月。此肿胀大小和数量逐渐增加。此外,她双侧肘部出现红色、瘙痒、隆起的皮肤病变,并逐渐蔓延至身体大部分部位,包括嘴唇、舌头和颊黏膜。这些皮肤病变导致吞咽液体和固体食物困难。包括活检在内的诊断检查确诊为PNP。随后,患者接受化疗及其他支持措施治疗,病情改善并最终出院。
PNP是一种与肿瘤相关的罕见水疱性疾病,常带来诊断和治疗挑战。PNP患者可能出现多种病变。及时识别并处理潜在恶性肿瘤对于改善患者预后至关重要。
本病例突出了T细胞淋巴瘤与PNP之间的罕见关联。临床医生对于非B细胞系淋巴瘤患者发生PNP的可能性也应保持警惕。