Onukogu Ifeanyichukwu, Ramachandran Preethi, Narh Joshua, Sahni Sonu, Joseph Gardith
Internal Medicine, Brookdale University Hospital and Medical Center, New York, USA.
Oncology, Brookdale University Hospital and Medical Center, Brooklyn, USA.
Cureus. 2020 May 27;12(5):e8316. doi: 10.7759/cureus.8316.
Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. An example is paraneoplastic pemphigus (PNP) which manifests as cutaneous bullae. Bullae may occur anytime during the course of the malignancy i.e. acute phase or remission. Diagnosis involves evaluation of clinical findings, serology and presence of characteristic histological findings. Its pathogenesis is described as development of auto-antibodies against cell junctional and basement membrane proteins. Presence of paraneoplasms has been associated with poorer prognosis and increased mortality in hematological malignancies including CLL. Currently, there are established indications for the treatment of CLL; however, presence of paraneoplasms as an indication for treatment is unclear. Patients with paraneoplasms who underwent expeditious treatment have exhibited better clinical outcomes. Herein we describe a case of a CLL patient in remission presenting with PNP and its response to treatment.
副肿瘤性疾病是与慢性淋巴细胞白血病（CLL）等血液系统恶性肿瘤相关的罕见多器官疾病。其中一些副肿瘤表现为皮肤病变，表现为单纯皮疹、溃疡或皮肤增厚。这一过程的发病机制被描述为针对细胞壁抗原和蛋白质产生某些自身免疫反应。一个例子是副肿瘤性天疱疮（PNP），其表现为皮肤大疱。大疱可能在恶性肿瘤病程的任何时候出现，即急性期或缓解期。诊断包括评估临床发现、血清学以及特征性组织学发现的存在情况。其发病机制被描述为针对细胞连接和基底膜蛋白产生自身抗体。副肿瘤的存在与包括CLL在内的血液系统恶性肿瘤的预后较差和死亡率增加有关。目前，CLL的治疗有既定的指征；然而，副肿瘤作为治疗指征尚不清楚。接受快速治疗的副肿瘤患者表现出了更好的临床结果。在此，我们描述一例缓解期CLL患者出现PNP及其对治疗的反应。
