Kasamatsu Hiroshi, Oyama Noritaka, Hasegawa Minoru, Oku Yohei, Inoue Genki, Kimura Makiko, Kanno Masataka, Kawakami Takumi, Ohta Hajime, Yoneshima Manabu
Department of Dermatology, Municipal Tsuruga Hospital, Tsuruga, Japan.
Department of Dermatology, Faculty of Medical Sciences, University of Fukui, Yoshida-gun, Japan.
J Dermatol. 2021 Feb;48(2):237-241. doi: 10.1111/1346-8138.15646. Epub 2020 Oct 18.
Pancreatic panniculitis (PP) is a rare clinical variant of subcutaneous fat necrosis, developing in patients with a variety of pancreatic diseases such as acute or chronic pancreatitis, tumors and cysts. The tumor-associated PP represents a noteworthy skin manifestation of underlying internal malignancies, also known as dermadrome. Among causative pancreatic tumors, acinar cell carcinoma is the most frequent malignancy; however, little is known about how the origin of tumor cells and progression stage of pancreatic tumors potentially contribute to the establishment of panniculitis. Here, we present a 69-year-old Japanese male case of clinically aggressive PP on the bilateral legs, whose skin lesions developed prior to the diagnosis of occult pancreatic tumor and liver metastasis. Moreover, the immunopathology of the pancreatic lesion revealed neuroendocrine tumor (NET), a rare pathological variant. Skin lesions immediately spread to the upper limbs with extensive ulcerations and necrosis, accompanied by high levels of serum lipase and elastase, but not with other pancreatic enzymes. He died 2 months after the initial development of the skin lesion due to rapid deterioration of general condition. We reviewed 14 cases, including ours, of PP with NET in the pancreas thus far reported, to identify the clinicopathological characteristics regarding to what extent this rare complication could reflect the clinical course of pancreatic tumors and overall prognosis. Our published work review found that the disease has a significant male predominance (male : female, 13:1) and cases with occult pancreatic tumors died within 4 months after the development of their skin lesions. Our case was the poorest prognostic outcome. This report emphasizes that dermatologists should recognize PP with NET, reflecting a fatal prognosis, and to make a prompt diagnosis.
胰腺性脂膜炎（PP）是皮下脂肪坏死的一种罕见临床变异型，发生于患有多种胰腺疾病的患者，如急性或慢性胰腺炎、肿瘤和囊肿。肿瘤相关性PP是潜在内部恶性肿瘤的一种值得注意的皮肤表现，也称为皮肤综合征。在引起PP的胰腺肿瘤中，腺泡细胞癌是最常见的恶性肿瘤；然而，关于肿瘤细胞的起源和胰腺肿瘤的进展阶段如何潜在地导致脂膜炎的发生，人们知之甚少。在此，我们报告一例69岁日本男性双侧腿部临床侵袭性PP病例，其皮肤病变在隐匿性胰腺肿瘤和肝转移诊断之前就已出现。此外，胰腺病变的免疫病理学显示为神经内分泌肿瘤（NET），这是一种罕见的病理变异型。皮肤病变迅速蔓延至上肢，伴有广泛溃疡和坏死，血清脂肪酶和弹性蛋白酶水平升高，但其他胰腺酶水平未升高。患者在皮肤病变最初出现后2个月因全身状况迅速恶化而死亡。我们回顾了包括我们的病例在内的迄今为止报道的14例胰腺NET合并PP病例，以确定这种罕见并发症在多大程度上能够反映胰腺肿瘤的临床病程和总体预后的临床病理特征。我们发表的文献综述发现，该疾病男性占显著优势（男∶女，13∶1），隐匿性胰腺肿瘤患者在皮肤病变出现后4个月内死亡。我们的病例预后最差。本报告强调皮肤科医生应认识到NET合并PP反映了致命的预后，并应及时做出诊断。
