皮肤浆细胞增多症和系统性浆细胞增多症

皮肤浆细胞增多症(cutaneous plasmacytosis,CP)的特征是躯干和四肢泛发红褐色斑块、结节和斑片,无任何自觉症状,常伴高γ-球蛋白血症.真皮可见多克隆成熟的浆细胞及淋巴细胞浸润;系统性浆细胞增多症(systemic plasmacytosis,SP)除有上述CP所有特征外尚累及2个或2个以上器官.该文对CP和SP的病因学、流行病学、临床分型及临床表现、组织病理改变、实验室检查、诊断和鉴别诊断、治疗和预后作一文献复习.     

Cutaneous and systemic plasmacytosis: a Chinese case

Cutaneous and systemic plasmacytosis (CSP) is an exceedingly rare condition arising primarily in patients of Japanese descent. Herein, we describe a patient of mainland Chinese origin suffering CSP. A 49-year-old Chinese male had asymptomatic brownish-red plaques and papules of the face and trunk for 6 years. Physical examination revealed innumerable symmetric red-brownish macules on face and trunk with fewer red-brownish papules scattered among the macules. Chemical analysis revealed hypergammaglobulinemia. Computerized tomography scan discovered some lymphadenopathy in the axillary, paratracheal and pulmonary regions. Histological examination showed focal perivascular and periadnexal infiltrate of mainly plasma cells in the superficial and deep dermis. Immunohistochemical study showed that a great number of the infiltrating cells were CD20-positive. The infiltrated polyclonal plasma cells expressed both kappa and lambda light chains. Topical therapy with tacrolimus 0.1% ointment for 2 months reduced the thickness and pigmentation of the facial skin lesions. The lesions resumed the original appearance 3 weeks after discontinuing the therapy. To the best of our knowledge, this is the first case of CSP from mainland China.     

儿童皮肤浆细胞增生症一例

患儿,女,8岁。因股部暗褐色斑块4年,于2017年1月来我院就诊。4年前,无明显诱因右股部内侧出现一蚕豆大红褐色斑块,无自觉症状,皮损渐增大至直径约4 cm的斑块。曾在当地外用多种药膏治疗,效果欠佳。现为求进一步诊治,遂来我院就诊。患者自发病以来神智清、精神可,饮食及睡眠如常。既往体健,否认系统疾病史,否认传染病史,否认药物及食物过敏史,否认家族其他人有类似病史,否认家族遗传病史。     

Cutaneous plasmacytosis limited to the extremities in a white patient: an unusual clinical picture

Cutaneous plasmacytosis is an uncommon disease characterized by a cutaneous polyclonal plasma cell infiltrate usually associated with polyclonal hypergammaglobulinemia. It has predominantly been found in Japanese patients and it is rare in white patients. Clinically, this condition manifests as multiple red to dark brown skin lesions that mainly are located on the trunk. We report the case of a 66-year-old white woman who presented with reddish brown to violaceous macules and plaques restricted to the extremities. The histopathologic findings, laboratory data, and systemic studies led us to the diagnosis of cutaneous plasmacytosis.     

Cutaneous and systemic plasmacytosis showing histopathologic features as mixed-type Castleman disease: a case report

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder characterized by disseminated reddish brown plaques and polyclonal hypergammaglobulinemia. The lesions of CSP are histologically characterized by an infiltration of mature polyclonal plasma cells, which display similar pathological features to the plasma cell-type Castleman disease (CD). The relationship between CSP and CD is controversial. Herein, we described a 43-year-old man from China with disseminated reddish brown plaques and nodules on the cheek and temple. The serum level of immunoglobulin G and immunoglobulin A were higher than normal. In addition to mature plasma cell perivascular infiltrate in the dermis, the biopsy of the lesions showed small to medium-sized germinal follicles with hyalinized vessels and a concentrically arranged mantle zone. The patient had clinical features of CSP, but the biopsy revealed changes resembling mixed-type CD. To the best of our knowledge, this is the first case of CSP with the pathological features of mixed-type CD reported from China.     

Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease

Cutaneous and systemic plasmacytosis is a rare condition primarily affecting middle‐aged individuals of Japanese descent and has principally been reported in cases originating from Asia. Its relationship, if any, to Castleman disease, is controversial. The authors report a case of cutaneous and systemic plasmacytosis involving a 36‐year‐old man of Chinese ancestry born in North America. The biopsy of an involved lymph node revealed changes resembling Castleman disease. Significantly, this case to our knowledge represents the first case of cutaneous and systemic plasmacytosis presenting in a patient of Chinese ancestry born on the North American mainland. Shadel BN, Frater JL, Gapp JDG, Hurley MY. Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease.     

Cutaneous plasmacytosis and multinucleate cell angiohistiocytoma‐like lesion in a patient with hepatitis B: A fortuitous triad?

A 28‐year‐old woman of Chinese descent, with congenital chronic hepatitis B presented with a 7‐year history of erythematous‐brown papules and plaques on her groins, axillae, and forehead. A first skin biopsy showed findings consistent with two concomitant, yet highly uncommon cutaneous diseases. The presence of lymphoid nodules with germinal centers and clustered polyclonal plasma cells was consistent with cutaneous plasmocytosis. Second, a diffuse proliferation of non‐atypical small vessels (CD31+, CD34+, and HHV8?) in a hypercellular stroma peppered with angulated giant cells (CD163+, CD68?) was suggestive of multinucleate cell angiohistiocytoma (MCAH). Interestingly, the second biopsy of a different plaque on the forehead showed only plasmacytosis and the clinical appearance of both plaques and papules alluded to the distinct presence of both concurrent entities. We speculate the immune modulating effects of chronic hepatitis B may have led to a polyclonal plasmacytic proliferation within the dermis. Furthermore, MCAH has been reported in conjunction with other inflammatory skin diseases such as hidradenitis suppurativa and as such we propose that the MCAH lesion in our case may have arisen as a secondary, reactive process to the cutaneous plasmacytosis.     

Cutaneous microbiologic profiles of Japanese adults residing in the United States of America

Quantitative cutaneous bacteriological profile analysis was performed on 20 healthy Japanese adults. Five ecologically different sites were cultured, the forehead, anterior nares, axilla, perineum, and fourth interspace. Antibiotic sensitivity patterns were determined for the various species of coagulase negative cocci (CNS). Aerobic organisms were found in densities exceeding 10(6) per sample for all sites except the forehead (10(4)), while anaerobic Propionibacteria were found in quantities ranging from 10(5) to 10(6) per sample except for the toe web space, which was devoid of anaerobic organisms. CNS were ubiquitous. Staphylococcus aureus varied from 15% to 35%. Lipophilic diphtheroids were found in 50% of the subjects on the forehead and nearly 100% on other sites, whereas multiple antibiotic resistant lipophilic diphtheroids (Group JK) were found in the perineum of 35% and in the toe space of 15%. Gram negative bacilli were uncommon on the forehead, axilla, and the toe web space, but were found in 50% or more in the anterior nares and perineum. Penicillin and ampicillin resistant CNS were commonly seen, while oxacillin and cephalosporin resistance were uncommon. Tetracycline and erythromycin resistance was seen in 35% to 40%.     

Cutaneous lesions as the first manifestation of systemic follicular lymphoma in an HIV patient

Human immunodeficiency virus (HIV) patients have an increased incidence of lymphomas, particularly when there is a significant immunosuppression. Most commonly, they are non-Hodgkin B cell type with a high or intermediate grade and have an extranodal presentation. We report the case of a 38-year-old man with HIV infection who presented with a 1-year history of a painless tumor on the back and lymphadenopathies. The diagnosis of B cell lymphoma follicle center cell type was established by skin biopsy. Staging included a bone marrow biopsy revealing infiltration by the lymphoma and a whole-body computed tomographic scan showing multiple cervical and axillary lymphadenopathies with necrotic center. Biopsy of an axillary lymph node revealed caseating epithelioid granulomas and Ziehl-Neelson staining was positive for acid-fast bacilli. The patient started therapy for tuberculosis and polychemotherapy for lymphoma with complete response. This report illustrates a case of simultaneous occurrence of tuberculosis and systemic follicular lymphoma presenting in skin in an HIV patient.     

Cutaneous leishmaniasis in a Senegal patient

Cutaneous leishmaniasis is a group of diseases with vast clinical polymorphism produced by protozoa of the genus Leishmania, that is acquired through the bite of sandflies. It is an endemic zoonosis in Spain, being the dog the main reservoir. In our country all forms of leishmaniasis are due to Leishmania infantum species, that usually produces mild lesions in uncovered areas, mainly in children. We report an imported case of cutaneous leishmaniasis in a Senegal patient that presented clinical characteristics unusually different from the typical lesions produced by L. infantum that we are used to evaluate. The lesions were multiple, large, very inflammatory and exudative; these differences may be attributed to the type of endemic leishmania in Senegal: L. major. Given the increase in immigrant population and travels abroad, it is essential for the dermatologist to become familiar with skin diseases of tropical areas that, in the near future, will be more common in daily clinical practice.     

Weight trends in United States living kidney donors: Analysis of the UNOS database

AIM: To analyze the national trends associated with body mass index (BMI) and living kidney donation.METHODS: Forty-seven thousand seven hundred and five adult living kidney donors as reported to the Organ Procurement and Transplantation Network from 1999 to 2011 were analyzed using their pre-donation BMI. Predictor variables of interest included age, gender, ethnicity, relationship, education status, and transplant region.RESULTS: Sixteen thousand nine hundred and seventy-one of the living kidney donors were normal weight (35.6%); 19337 were overweight (40.5%); 9007 were mildly obese (18.9%); 1992 were moderate to morbidly obese (4.2%). Overweight and mildly obese kidney donors have increased through time by 12% and 20% every 5 years, respectively (P < 0.05). Donors 35-49 years of age, hispanic males or females and black females, those with high school diploma or general Education Degree, and biologically related or partner/spouses were more likely to be obese.CONCLUSION: Over the past 13 years, the majority of living kidney donors have spanned the overweight to obese categories. Paralleling the national rise is an increase in overweight and mildly obese kidney donors. A fair number of moderate to morbidly obese living kidney donors are still allowed to donate.