Cutaneous and Systemic Plasmacytosis Associated with Renal Amyloidosis

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder of unknown etiology characterized by cutaneous polyclonal plasma cell infiltrates associated with various extracutaneous involvement and polyclonal hypergammaglobulinemia. Here, we report on a 54-year-old male patient with chronic renal insufficiency who presented with disseminated reddish-brown macules and plaques on the face and trunk. In our evaluation, he was found to have lymphadenopathy, polyclonal hypergammaglobulinemia; benign plasma cell infiltration involving the skin, bone marrow, and retroperitoneal area; and renal amyloidosis. To the best of our knowledge, this is the first reported case of CSP associated with renal amyloidosis.     

皮肤浆细胞增生症1例及相关文献复习

皮肤浆细胞增生症是一种罕见的疾病,其特征主要是皮肤良性多克隆浆细胞浸润,通常与多克隆γ-球蛋白血症相关.其典型的临床表现为红褐色斑、斑块或结节,高γ-球蛋白血症,一半以上的患者伴有不同程度的淋巴结病.据以往文献报道,此病主要发生于日本中老年人群.截止到目前为止,关于中国人皮肤浆细胞增生症的报道尚少.在此报道52岁的中国女性浆细胞增生症1例,并复习以往相关文献,对其流行病学、临床特征、病理特点、病因病机、诊断、治疗及预后等进行总结分析.     

皮肤浆细胞增多症1例

报告１例皮肤浆细胞增多症，患者女，３２岁，额、面、躯干、四肢散布暗红色至淡褐色斑疹、斑块、直径０．５￣１．５ｃｍ。系统检查无异常。实验室检查发现血沉升高，α２－，β－，γ－球蛋白轻度升高。皮损活检组织病理示真皮血管周围密集浆细胞浸润，这些浆细胞成熟，无异型。     

Cutaneous plasmacytosis associated with lung and anal carcinomas

Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobulinaemia. We present the case of a 55-year-old Caucasian man who suffered from a cutaneous plasmacytosis associated with two different carcinomas. Cutaneous plasmacytosis seems to be a reactive process because most cases reported are not associated with any apparent underlying disease. Nevertheless, because few reported cases were associated with malignancies, screening of additional neoplasms would be justified.     

Treatment of cutaneous plasmacytosis with mask‐bath PUVA: A therapeutic option

Cutaneous plasmacytosis (CP) is an uncommon chronic disease of unknown aetiology, reported mainly in middle‐aged patients of Asian descent. It is diagnosed by a constellation of physical, laboratory, radiological and histopathological findings. We report a patient with CP who demonstrated a favorable and promising response to mask‐bath PUVA.     

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.     

A Case of Necrobiosis Lipoidica Treated with Systemic Corticosteroid

A 43-year-old woman had asymptomatic eruptions on the anterior sides of both legs for 5 years. Biopsy specimens revealed the typical findings of necrobiosis lipoidica. An immunofluorescence study demonstrated no deposits of immunoglobulins, complement, or fibrinogen in vessel walls. Immunohistochemical staining revealed CD4 positive lymphocytes dominating in the lesion and strong expression of ICAM-l on the endothelial cells, giant cells, lymphocytes and dermal fibroblasts in the lesion. The eruptions resolved remarkably with oral corticosteroid therapy.     

白癜风合并系统性硬皮病1例

患者男,75岁。全身皮肤白斑25年,四肢躯干皮肤变硬2年,双下肢红斑、丘疹、糜烂、渗出、结痂4月,诊断为白癜风、硬皮病、湿疹。     

皮肤浆细胞增多症

报告1例皮肤浆细胞增多症.患者男,51岁.右下肢股外侧浸润性红斑4年余,口周和腹部皮损1年.皮损组织病理检查:真皮深层及部分皮下组织可见以成熟浆细胞为主的大量炎性细胞浸润,其间有少量淋巴细胞及多核巨细胞.免疫组化染色示:浸润的浆细胞CD79a、CD138阳性.诊断:皮肤浆细胞增多症.     

Case of isolated benign primary cutaneous plasmacytosis in a child

A collection of plasma cells in the skin can represent a broad spectrum of disease entities. Secondary syphilis, primary cutaneous plasmacytoma, primary cutaneous plasmacytosis, cutaneous lymphoid hyperplasia and nodular amyloidosis are considered possible differential diagnoses. We present a case of a 7-year-old girl with an erythematous scaly plaque on her right buttock that had been present for approximately 5 years. Prior to her visit to our department she had been treated at a local dermatology clinic with topical methylprednisolone acetate and topical calcitriol without significant improvement. Histopathological examination revealed psoriasiform hyperplasia, hyperkeratosis, parakeratosis and a band-like or dense perivascular infiltration of plasma cells with a few lymphocytes and histiocytes. Other laboratory tests were within the reference ranges. At our department, the patient was given oral prednisolone along with an intralesional injection of triamcinolone and application of topical methylprednisolone acetate and tacrolimus hydrate to the affected area. The lesion improved significantly but recurred 3 months later. We present a rare case of isolated benign primary cutaneous plasmacytosis in a female pre-adolescent child.     

原发性系统性淀粉样变病1例

患者男,69岁。躯干部散在瘀斑1年余。皮肤科情况:面部、颈部、躯干部、双上肢伸侧散在大小不等紫红色斑,压之不退色,颈部、双上肢伸侧可见紫红色结节,有蜡样光泽,触之质韧。骨髓检查示骨髓增生低下,浆细胞比值增高,占7%。皮肤组织病理检查示:真皮浅层血管周围大量均一红染物质,刚果红染色阳性。诊断:原发性系统性淀粉样变病。     

大疱性系统性红斑狼疮1例

患者女,38岁。确诊为系统性红斑狼疮1年。近3周来,全身出现粟粒大紧张或松弛性水疱,尼氏征(-),皮损组织病理示:表皮下疱形成,直接免疫荧光示:IgA,IgG,IgM和C3线状沉积于基底膜带。诊断:大疱性系统性红斑狼疮。予泼尼松和沙利度胺治疗2周后,症状已被控制。     

大疱性系统性红斑狼疮1例

报告1例大疱性系统性红斑狼疮。患者女,22岁。2009年5月逐渐出现头痛、关节痛、牙龈出血、呕血、周身瘀点、瘀斑、血小板减少。此次因口腔反复溃疡4月余,周身红斑水疱1月余,视物不清1周就诊。临床表现为红斑、水疱、口腔溃疡。实验室检查符合SLE诊断。背部水疱处组织病理检查示:表皮下水疱形成,内含中性粒细胞、淋巴细胞及纤维素网,基底细胞液化变性。毛囊周围较多淋巴细胞浸润。直接免疫荧光显示IgG,C3基底膜带线状荧光沉积。     

Case of recalcitrant necrobiotic xanthogranuloma

A 53-year-old woman with necrobiotic xanthogranuloma presented as infiltrated eyelid plaques, with later development of arm and thigh lesions. Clinical features, including association with immunoglobulin G-kappa paraprotein, and pathological findings were typical of this disorder. Treatment for 15 months with varying combinations of prednisone and multiple chemotherapeutic agents (melphalan, cyclophosphamide and chlorambucil) has led to minimal or no improvement in the clinical lesions or paraprotein. The case demonstrates some of the difficulties in managing this unusual disorder.     

疣状表皮发育不良继发鳞状细胞癌1例

患者男,34岁。躯干、四肢出现扁平丘疹20年,面部肿块、结节1年余。皮肤组织病理示丘疹性损害,表皮颗粒层细胞呈程度不等的不规则肥厚和空泡变性;结节性损害:鳞状细胞瘤块由表皮不规则向真皮增生,细胞异形性改变,核分裂活跃,角珠形成,瘤块周围炎性细胞浸润。     

儿童大疱性系统性红斑狼疮1例

患儿女 ,10岁。因关节疼痛伴血尿 2月 ,全身皮肤起水疱 1月余入院。入院后查抗核抗体阳性 ,抗sm抗体阳性 ;皮损组织病理示表皮下疱 ;直接免疫荧光示基底膜带IgG ,IgA ,IgM ,C3 呈线状沉积。诊断为儿童大疱性系统性红斑狼疮。     

A Case of Progressive Systemic Sclerosis Associated with Sarcoidosis and Esophageal Adenocarcinoma

A 50-year-old Japanese female with progressive systemic sclerosis (PSS, CREST syndrome) is reported. During treatment for PSS, she was diagnosed by clinical and laboratory findings as having sarcoidosis, which was confirmed by histological examination of the skin and lymph nodes in July of 1991. She complained of back pain in August of 1991. Reflux esophagitis and Barrett's esophagus, found by endoscopy, progressed into a well-differentiated tubular adenocarcinoma. This is a very rare case of PSS associated with sarcoidosis and esophageal adenocarcinoma developing from Barrett's esophagus.