Gao Yinjie, Cui Yunying, Hu Zhonghui, Wang Yu, Li Tianyi, Liu Yuehua, Tong Anli
NHC Key Laboratory of Endocrinology, Peking Union Medical College Hospital, Beijing, China.
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Front Med (Lausanne). 2022 Nov 17;9:1065350. doi: 10.3389/fmed.2022.1065350. eCollection 2022.
Tumor-associated cutaneous vascular disorder induced by PPGL was extremely rare, and the cutaneous manifestations could disappear after removal of the tumors. However, the definite pathological diagnosis and the potential mechanism remained unidentified. We presented a severe cutaneous vascular lesion manifested as diffuse erythema with ulceration and necrosis over the limbs in a female patient with metastatic paraganglioma. Skin biopsy was performed on her for defining the pathological diagnosis and potential mechanism. The patient was diagnosed as vascular disease according to the obvious angioectasia in dermis on cutaneous pathology, which might be caused by PPGL-induced hypercoagulability. We used the antiplatelet therapy with aspirin to treat the PPGL-associated cutaneous vascular disease for the first time, and the cutaneous lesions were relieved and healed gradually, further supporting the diagnosis of vascular disease.
For metastatic PPGL patients like the case we reported, the definite diagnosis by skin biopsy and the early antiplatelet therapy might be effective to the cutaneous lesions caused by the hypercoagulable state of PPGL.
由嗜铬细胞瘤/副神经节瘤(PPGL)引起的肿瘤相关性皮肤血管疾病极为罕见,且在肿瘤切除后皮肤表现可能会消失。然而,确切的病理诊断和潜在机制仍不明确。我们报告了一名患有转移性副神经节瘤的女性患者,其四肢出现严重的皮肤血管病变,表现为弥漫性红斑伴溃疡和坏死。对她进行了皮肤活检以明确病理诊断和潜在机制。根据皮肤病理学检查发现真皮内明显的血管扩张,该患者被诊断为血管疾病,这可能是由PPGL诱导的高凝状态所致。我们首次使用阿司匹林进行抗血小板治疗来治疗与PPGL相关的皮肤血管疾病,皮肤病变逐渐缓解并愈合,进一步支持了血管疾病的诊断。
对于像我们报道的这种转移性PPGL患者,通过皮肤活检进行明确诊断以及早期抗血小板治疗可能对由PPGL高凝状态引起的皮肤病变有效。