PDF(Pubmed)
Abstract：
UNASSIGNED: Adenopathy and extensive skin patch overlying plasmacytoma syndrome is a paraneoplastic syndrome characterized by a cutaneous vascular patch overlying a plasmacytoma and systemic manifestations. It is thought to be an early stage of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome, which is a rare, but potentially fatal multisystemic disease that is associated with plasma cell dyscrasia. Thus, a high index of suspicion is required to identify patients with adenopathy and extensive skin patch overlying plasmacytoma as they may present with early polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, which is curable if detected early.
UNASSIGNED: To report additional cases of adenopathy and extensive skin patch overlying plasmacytoma syndrome, describe dermatoscopic and histologic findings of the cutaneous patch and review all up to date literature on adenopathy and extensive skin patch overlying plasmacytoma syndrome.
UNASSIGNED: Case series from a single tertiary care center.
UNASSIGNED: Here, we present the second case series of three patients with adenopathy and extensive skin patch overlying plasmacytoma syndrome who all meet the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The diagnosis was suspected based on the presence of the violaceous cutaneous patch along with symptoms of systemic involvement (fatigue, weight loss, weakness). Dermoscopy revealing regular dilated parallel capillaries was suggestive of a benign/reactive vascular process. Histopathology in all three cases showed reactive vascular proliferation with a characteristic 90° branching. To date only 20 cases of adenopathy and extensive skin patch overlying plasmacytoma have been published, including ours. All patients presented with cutaneous lesions (violaceous patch and others) and most, at least 15/20, met the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. When clinical follow-up was reported, most patients had a favorable prognosis with partial or complete symptom resolution following treatment of the underlying plasmocytoma.
UNASSIGNED: To report additional cases of adenopathy and extensive skin patch overlying plasmacytoma syndrome, describe dermatoscopic and histologic findings of the cutaneous patch and review all up to date literature on adenopathy and extensive skin patch overlying plasmacytoma syndrome.
UNASSIGNED: Case series from a single tertiary care center.
UNASSIGNED: Here, we present the second case series of three patients with adenopathy and extensive skin patch overlying plasmacytoma syndrome who all meet the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The diagnosis was suspected based on the presence of the violaceous cutaneous patch along with symptoms of systemic involvement (fatigue, weight loss, weakness). Dermoscopy revealing regular dilated parallel capillaries was suggestive of a benign/reactive vascular process. Histopathology in all three cases showed reactive vascular proliferation with a characteristic 90° branching. To date only 20 cases of adenopathy and extensive skin patch overlying plasmacytoma have been published, including ours. All patients presented with cutaneous lesions (violaceous patch and others) and most, at least 15/20, met the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. When clinical follow-up was reported, most patients had a favorable prognosis with partial or complete symptom resolution following treatment of the underlying plasmocytoma.
摘要：
未经证实：腺病和广泛皮肤斑块覆盖浆细胞瘤综合征是一种副肿瘤综合征，其特征是皮肤血管斑块覆盖浆细胞瘤和全身表现。这被认为是多发性神经病的早期阶段，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤改变综合征，这是一种罕见的，但可能致命的多系统疾病与浆细胞发育不良有关。因此，高度怀疑是必要的，以确定患者的腺病和广泛的皮肤斑块覆盖浆细胞瘤，因为他们可能存在早期多发性神经病，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤的变化，如果早期发现是可以治愈的。
未经证实：报告更多的腺病和广泛的皮肤斑块覆盖浆细胞瘤综合征病例，描述皮肤贴片的皮肤镜和组织学发现，并回顾所有关于腺病和覆盖浆细胞瘤综合征的广泛皮肤贴片的最新文献。
UNASSIGNED：来自单一三级护理中心的病例系列。
未经评估：这里，我们介绍了第二例病例系列，包括3例腺病和广泛的皮肤斑块覆盖浆细胞瘤综合征患者，他们都符合多发性神经病的诊断标准，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤变化。怀疑诊断是基于皮肤皮肤皮肤的存在以及全身受累的症状（疲劳，减肥,弱点)。皮肤镜检查显示规则扩张的平行毛细血管提示良性/反应性血管过程。所有三例病例的组织病理学均显示反应性血管增生，特征性90°分支。迄今为止，仅发表了20例腺病和覆盖浆细胞瘤的广泛皮肤贴片，包括我们的.所有患者均出现皮肤病变（紫红色斑块和其他），大多数患者，至少15/20，符合多发性神经病的诊断标准，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤变化。当临床随访报告时，大多数患者在治疗基础浆细胞瘤后预后良好,症状部分或完全缓解.
未经证实：报告更多的腺病和广泛的皮肤斑块覆盖浆细胞瘤综合征病例，描述皮肤贴片的皮肤镜和组织学发现，并回顾所有关于腺病和覆盖浆细胞瘤综合征的广泛皮肤贴片的最新文献。
UNASSIGNED：来自单一三级护理中心的病例系列。
未经评估：这里，我们介绍了第二例病例系列，包括3例腺病和广泛的皮肤斑块覆盖浆细胞瘤综合征患者，他们都符合多发性神经病的诊断标准，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤变化。怀疑诊断是基于皮肤皮肤皮肤的存在以及全身受累的症状（疲劳，减肥,弱点)。皮肤镜检查显示规则扩张的平行毛细血管提示良性/反应性血管过程。所有三例病例的组织病理学均显示反应性血管增生，特征性90°分支。迄今为止，仅发表了20例腺病和覆盖浆细胞瘤的广泛皮肤贴片，包括我们的.所有患者均出现皮肤病变（紫红色斑块和其他），大多数患者，至少15/20，符合多发性神经病的诊断标准，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤变化。当临床随访报告时，大多数患者在治疗基础浆细胞瘤后预后良好,症状部分或完全缓解.
