Primary cutaneous plasmacytosis in a child. Is this a new entity?

Plasma cell proliferations represent a heterogeneous spectrum of disorders. A 7-year-old Caucasian female had suffered an asymptomatic eruption on the trunk for 4 years. Physical examination revealed a plaque with scattered red-brown papules and nodules. Chemical analysis revealed normal proteinaemia. Histological examination of biopsy specimens showed dense perivascular and periadnexal infiltrate, consisting largely of plasma cells, in the superficial and deep dermis. Immunohistochemical study showed that many cells of the infiltrate were CD20 positive. The plasma cells expressed kappa and lambda light chains. The girl's status (age; absence of hypergammaglobulinaemia, lymphadenopathy and hepatosplenomegaly; presence of an infiltrate of mature polyclonal plasma cells restricted only to the skin) differed from those generally seen in diseases with plasma cell proliferation reported in the literature. This case seems unlike any other described up to the present time.     

皮肤及系统性浆细胞增多症一例

患者男,46岁。6年余前背部出现散在红褐色斑块和结节,逐渐发展至腋窝及前胸部,质地变硬,冬季稍瘙痒。实验室检查发现高丙种球蛋白血症。电子计算机断层扫描（CT）发现双肺多发结节、斑片灶,纵隔、腋窝及腹股沟淋巴结肿大,脾脏增大。皮损组织病理检查：真皮中下层可见大量淋巴细胞、组织细胞、成熟浆细胞形成的肉芽肿性浸润,并可见淋巴滤泡样结构,未见细胞异形;免疫组化染色：CD38、CD138、CD79a、κ轻链、λ 轻链均阳性。结合临床及实验室检查,诊断为皮肤及系统性浆细胞增多症。     

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.     

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.     

BCL2 and JUNB abnormalities in primary cutaneous lymphomas

BACKGROUND: BCL2 is upregulated in nodal and extranodal B-cell non-Hodgkin's lymphomas, with a consequent antiapoptotic effect. However, loss of BCL2 has also been noted in some malignancies, suggesting a different molecular pathogenesis. OBJECTIVES: To investigate genomic and protein expression status of BCL2 and to compare the results with that of JUNB in primary cutaneous lymphomas (PCLs). METHODS: We analysed gene copy number of BCL2 and JUNB in 88 DNA samples from 80 patients with PCL consisting of Sézary syndrome/mycosis fungoides (SS/MF), primary cutaneous B-cell lymphoma (PCBCL) and primary cutaneous CD30+ anaplastic large cell lymphoma (C-ALCL) by the use of real-time polymerase chain reaction (PCR) and immunohistochemistry (IHC). Real-time PCR and IHC findings were subsequently compared with the results of additional fluorescent in situ hybridization (FISH) analysis of 23 cases of SS and Affymetrix cDNA expression microarray study of two primary cutaneous T-cell lymphoma (CTCL) cell lines. RESULTS: Real-time PCR analysis showed loss of BCL2 gene copy number in 22 of 80 PCL cases (28%), including 17 of 42 SS/MF, three of 13 C-ALCL and two of 33 PCBCL samples, and gain of BCL2 in four PCBCL samples. Gain of JUNB was identified in 18 of 71 PCL cases (25%), including nine of 35 SS/MF, seven of 13 C-ALCL and two of 31 PCBCL samples. IHC analysis revealed absent nuclear expression of BCL2 protein in 47 of 73 PCL cases, comprising 28 of 36 SS/MF, eight of eight C-ALCL and 11 of 29 PCBCL cases. In contrast, BCL2 protein expression was detected in 26 of 73 PCL cases, consisting of 18 of 29 PCBCL and eight of 36 SS/MF cases. JUNB protein expression was present in tumour cells from 30 of 33 of SS/MF and eight of eight C-ALCL, and was absent in tumour cells from 18 of 27 PCBCL cases. A comparison between BCL2 and JUNB revealed loss of BCL2 and gain of JUNB in five of 35 SS/MF samples, and expression of JUNB protein and absent BCL2 expression in 25 SS/MF and eight of eight C-ALCL cases. In contrast, expression of BCL2 and absent JUNB expression were detected in 67% of PCBCL cases. Additional FISH analysis revealed deletion of BCL2 in 19 of 23 SS cases (83%), including eight cases with BCL2 loss shown by real-time PCR. Furthermore, Affymetrix expression microarray demonstrated decreased expression of proapoptotic and antiapoptotic genes involved in BCL2 signalling pathways such as BOK, BIM, HRK, RASA1 and STAT2 in two CTCL cell lines with BCL2 loss and absent BCL2 expression. Increased expression of JUNB was also identified in the MF cell line. CONCLUSIONS: These findings provide a comprehensive assessment of BCL2 and JUNB status in PCL, and suggest that there is a selection pressure in a subset of CTCL cases for tumour cells showing BCL2 loss and upregulation of JUNB primarily through chromosomal deletion and amplification, respectively.     

Frequent immunoglobulin G4 expression in a common variant of primary cutaneous marginal zone B‐cell lymphoma

Immunoglobulin (Ig)G4 expression was recently observed in a proportion of primary cutaneous marginal zone B‐cell lymphoma (PCMZL) with plasmacytic differentiation. IgG4‐related disease is characterised by polyclonal lymphoplasmacytic infiltration with IgG4 expression, storiform fibrosis and obliterative phlebitis in histopathology. Here we report three cases of common variants of PCMZL with predominant and varied IgG4 expression, suggesting there is an underlying clonal progression between these two entities.     

原发性皮肤间变性大细胞淋巴瘤

报告1例原发性皮肤间变性大细胞淋巴瘤.患者女,24岁.左手中指红斑33 d,根据皮损组织病理及免疫组化标记结果诊断为原发性皮肤间变性大细胞淋巴瘤.     

Case of primary cutaneous peripheral T‐cell lymphoma,not otherwise specified,with characteristics of follicular helper T cells

We report a case of an 88‐year‐old woman with a decalvant, erythematous, ulcerated tumor extending from the right temporal to occipital region. Histopathological analysis revealed a dense infiltration of medium‐to‐large‐sized atypical cells throughout the entire dermis. The result of immunohistochemical analysis showed that the infiltrating T cells expressed programmed death‐1 (PD‐1), Bcl‐6 and CXCL13. Flow cytometry analysis showed that CD4⁺ PD‐1^hi T cells also expressed CD10, inducible T‐cell co‐stimulator and CXCR5. On the basis of the clinical appearance and the histopathological findings, we diagnosed the patient with primary cutaneous peripheral T‐cell lymphoma, not otherwise specified. Recently, the concept of primary cutaneous follicular helper T (TFH)‐cell lymphoma was proposed, and in this case, tumor cells clearly expressed TFH‐cell markers. Therefore, we considered this case to be a variant of the entity. Although this entity is still provisional, this case supports the new concept.     

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy of the skin and hematopoietic system. There are few pediatric cases reported in the literature. Management of primary cutaneous BPDCN is challenging because, despite an apparently indolent clinical presentation, rapid dissemination with high mortality can occur. We describe a child with isolated cutaneous involvement who had a good response to chemotherapy as first‐line treatment of BPDCN.     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

原发性皮肤隐球菌病一例

报告1例自身免疫功能正常的原发性皮肤隐球菌病。患者因右腋窝肿块、进行性加重8个月就诊。皮肤科情况:右腋窝可见10 cm×2.6 cm隆起性肿块,质软、稍有波动感,边界清楚,活动度差;额部、前胸部散在米粒至黄豆大小丘疹及结节。根据其临床症状、组织病理检查、特殊染色及真菌学检查,确诊为原发性皮肤隐球菌病。经局部手术切除及口服伊曲康唑治疗痊愈。     

原发性皮肤浆细胞瘤1例

报告1例原发性皮肤浆细胞瘤。患者男,76岁。初发为颌部单发的无痛性结节,后发展为多发损害。组织病理检查及免疫组化染色结果提示真皮全层及皮下脂肪克隆性浆细胞增生。患者病程近10年,长期随访及多次系统检查未发现系统受累症状,诊断原发性皮肤浆细胞瘤,给予VIP（M：骒法仑,P：泼尼松）方案化疗,取得较好的效果。     

Electrochemotherapy as a novel treatment for primary cutaneous marginal zone B‐cell lymphomas

In the present study, we describe the use of electrochemotherapy as alternative therapy for primary cutaneous marginal zone B‐cell lymphomas in patients unsuitable for surgery or radiotherapy. Our experience refers to three patients with primary cutaneous marginal zone B‐cell lymphomas related to Borrelia burgdorferi infection, treated with specific antimicrobial therapy and electrochemotherapy.     

儿童面中部NK/T细胞淋巴瘤合并曲霉感染1例

报告儿童面中部NK/T细胞淋巴瘤合并曲霉感染1例。患儿女，8岁，左鼻部皮损6个月，伴发热、左面部肿胀5个月。检查见左务翼组织表面有污褐色痂，周围糜烂，有脓性分泌物，并有异味，周围组织水肿，口腔黏膜有溃疡及坏死。肝脾大。末梢血全血细胞降低。创面镜检邮大量分隔菌丝，经培养鉴定为曲霉菌属。组织病理检查真皮至皮下脂肪有密集的淋巴细胞浸润。真皮血管周围有散在异形的淋巴细胞，皮下脂肪层有吞噬红细胞的组织细胞。免疫组化染色示CD45RO（ ），散在CD56（ ）。PAS染色可见菌丝和孢子。     

原发性结节型皮肤淀粉样变性

报告1例原发性结节型皮肤淀粉样变性。患者男,41岁,双侧鼻孔缘下斑块、结节4年就诊,无自觉症状。组织病理检查:表皮基底细胞轻度液化变性,真皮浅、深层及皮下组织胶原纤维间可见嗜酸性物沉积,血管壁见类似改变。结晶紫和刚果红染色阳性,PAS染色弱阳性,偏振光显微镜下呈苹果绿双折光。诊断:原发性结节型皮肤淀粉样变性。     

Two cases of primary hyperparathyroidism associated with primary cutaneous lymphoma

Primary hyperparathyroidism has been described previously in association with malignancy, but to our knowledge has not been reported in association with primary cutaneous lymphoma. We report two cases of parathyroid adenoma with primary cutaneous lymphoma, the first in a 42-year-old woman with CD30-negative cutaneous large cell lymphoma, and the second in a 67-year-old man with mycosis fungoides and CD30-positive anaplastic large cell lymphoma.     

原发性皮肤淀粉样变一家系OSMR基因突变检测

目的: 明确原发性皮肤淀粉样变（PCA）的致病基因抑瘤素M受体（OSMR）突变情况。方法：提取一PCA家系3例患者及22名正常成员的外周血DNA,应用PCR扩增OSMR基因外显子,并对产物进行测序。 结果：该家系3例患者OSMR基因的第15外显子中均检测出c.2081C>T（p.Pro694Leu）错义突变,而家系内正常人未见该突变。结论：该家系患者发病可能与OSMR基因第15外显子突变有关。     

A case of primary cutaneous histoplasmosis in a patient with diabetes and multi-infarct dementia

We report a case of primary cutaneous histoplasmosis in a fifty-year-old African-American woman with diabetes and multi-infarct dementia. The patient developed fever and crusted, nodulo-ulcerative lesions of the skin after accidental superficial trauma to the forehead. The biopsy revealed suppurative granulomatous inflammation with intracellular and extracellular yeast-like cells with associated clear halo measuring 3-4 mm in size. Systemic involvement was not found. The lesions cleared after treatment with itraconazole 200 mg twice a day for 3 weeks. The medication was continued for a total period of 3 months, with no signs of recurrence after one-year of follow-up.