原发性皮肤中心母细胞淋巴瘤1例

报告１例原发性皮肤中心母细胞淋巴瘤。患者,男,９２岁。右小腿被蚊虫叮咬后出现肿块１年半,增多、扩大、破溃伴左小中块２个月。血常规、Ｘ光胸片、腹部Ｂ超、骨髓检查未划常。病检示肿瘤细胞形态似中心母细胞,免疫组化证实瘤细胞的Ｂ细胞性。ＥＢＥＲ原位杂交末检测到ＥＢ病毒。患者未作放化疗,９个月后死亡。     

T-zone lymphoma with cutaneous involvement: a case report and review of the literature

T-zone lymphoma (TZL) is a rare subtype of nodal peripheral T-cell lymphoma characterized by a clonal expansion of T-zone lymphocytes accompanied by a proliferation of other T-zone constituents. Non-specific cutaneous alterations are seen in about one-third of all cases, but specific cutaneous involvement is extremely rare. We present a case of TZL with secondary skin infiltration, review the literature on cutaneous manifestations of TZL and discuss the differential diagnosis of TZL.     

皮肤血管内T细胞淋巴瘤一例

患者女,64岁,6个月前全身出现多发淡红色斑丘疹,质硬,直径1～3 cm,以左侧颜面部及双下肢明显.曾就诊于某医院,诊断为血管炎.1个月前出现头痛、头晕伴步态不稳,同时伴双手及左足麻木、视物模糊,行头颅CT检查提示"右侧额辐射区腔隙性脑梗塞",1个月来步态不稳逐渐加重,再次就诊于当地医院,行头颅MRI检查提示"双侧丘脑、右侧基底节、右侧额叶及右侧小脑半球梗塞,左侧蝶窦旁异常信号灶".患者为进一步诊治于2010年1月至本院治疗,门诊拟诊脑梗死收住入院.皮疹予复方倍他米松注射液、冰黄肤乐软膏等治疗后稍见好转.     

原发性皮肤弥漫性大B细胞淋巴瘤（腿型）二例并文献复习

弥漫性大B细胞淋巴瘤（腿型）是皮肤B细胞淋巴瘤的一个罕见亚型，具有特征性的临床、病理、免疫组化及演变特征。本文报道2例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）并复习相关文献。     

Angioimmunoblastic T‐cell lymphoma with a clonal plasma cell proliferation that underwent immunoglobulin isotype switch in the skin,coinciding with cutaneous disease progression

Plasma cell proliferations in specific cutaneous lesions of angioimmunoblastic T‐cell lymphoma(AITL) are very uncommon. Here, we report a case of clonal plasma cell proliferation in skin with heavy‐chain‐immunoglobulin‐isotype‐switch after cutaneous disease progression. Histopathologically, initial plaque lesions were suggestive of marginal‐zone B‐cell‐lymphoma. Nevertheless, this 77‐year‐old lady was diagnosed with AITL after the progression of skin lesions from plaques to nodular tumors. A lymph node biopsy confirmed the diagnosis. Both cutaneous specimens showed a polymorphic cellular infiltrate with atypical T‐cell‐lymphocytes arranged in a pseudonodular pattern that expressed CD3, PD1 and BCL6, with patchy expression of CD30. Interestingly, a slight IgG‐Lambda plasma cell component was seen at the periphery of the infiltrate in the first specimen which increased in number in the later nodular lesion, showing not only Lambda light chain restriction and IgG but also IgG4. PCR studies for IgH and TCR genes showed an IgH clonal peak on both skin lesions but not on lymph node biopsy. On the contrary, the same clonal TCR peak was found in the three specimens. Neoplastic follicular helper T‐cells within cutaneous‐specific microenvironment could be responsible for the modulation of the immunoglobulin isotype class switch change. Further studies are needed to support this hypothesis.     

The cutaneous pathology of lupus erythematosus: a review

The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti-Ro/SSA-positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19-associated LE.     

CD30 (Ki-l)-positive primary cutaneous T-cell lymphoma: Report of spontaneous resolution

A 28-year-old man in whom CD30 (Ki-1)-positive cutaneous large cell lymphoma manifested as multiple widespread ulcerated nodules, which developed over a 2-week period, is reported. The lymphoma was composed of large anaplastic cells in large clusters, with scattered eosinophils, neutrophils and small lymphocytes. Extensive staging investigations revealed no extra-cutaneous disease. No treatment was given because, within weeks of presentation, the nodules began to regress spontaneously and the patient remains disease-free 20 months later.     

Primary cutaneous B cell lymphoma: Outcomes and treatment

This retrospective study documents six patients with primary cutaneous follicular centre cell lymphoma (FCCL) of the head and trunk. The hack was the most common site of presentation of the primary. Despite a good response to initial therapy, cutaneous relapses were common and one patient developed lymph node metastases. Intralesional steroids may be effective in controlling localized skin relapses of this B cell lymphoma. All patients are currently alive with two surviving over 5 years.     

以大疱表现为主的小儿B细胞淋巴瘤1例

报告１例特异性皮疹表现为红斑、大疱的Ｂ细胞淋巴瘤。患儿男性，３岁，皮疹表现为全身泛发的红斑以及红斑基础上的大疱，疱壁紧张，疱液清，尼氏征（－）。病程中第一次组织病理检查可见多数表皮下水疱，未见斑块状密集的单一核细胞浸润于皮下及真皮附属器周围。免疫病理检查阴性。２８天后作皮肤和淋巴结组织病理检查及免疫组化证实为ＣＢＣＬ。     

原发性皮肤小B细胞淋巴瘤

报告1例原发性皮肤小B细胞淋巴瘤。患者男，62岁。反复低热4年，躯干暗红斑、斑匠疹1年。外周血白细胞和淋巴细胞计数正常，IgM56100mg／L，κ轻链（free κ）21，73g/k尿本-周蛋白阳性。3次骨髓穿刺均显示增生性骨髓像。皮损组织病理检查：真皮层及胶原束间密集淋巴样细胞浸润，免疫组化染色结果示肿瘤细胞CD20、CD79a、CD5、CD10、部分CD23、bcl-2均（＋），UCHL1、CD4、CD8、CD38、cyclin D1均（-）。组织病理改变符合小B细胞淋巴瘤。诊断：原发性皮肤小B细胞淋巴瘤。     

继发性皮肤浆细胞瘤

报告1例继发于多发性骨髓瘤（multiple myelonm,MM)的皮肤桨细胞瘤，患者女，44岁，皮损为多发性，来源于MM直接蔓延和血行转移，组织病理检查示瘤细胞呈结节状浸润，免疫组化染色结果示IgG阳性表达，患者于皮损出现后78d死亡。     

Primary cutaneous angioplasmocellular hyperplasia

Primary cutaneous angioplasmocellular hyperplasia is a plasmacellular infiltrate that has been reported only once previously in the literature, in a report of a case affecting two Latin American patients. In the present case, a systemically well 62-year-old Caucasian man presented with a nodule on the back of the neck. Histology showed a vascular proliferative process with an abundance of plasma cells in the stroma. There has been no recurrence of the lesion 2 years following curettage and diathermy. The clinicopathological presentation is consistent with primary cutaneous angioplasmocellular hyperplasia.     

Nasal natural killer/T cell lymphoma with cutaneous involvement: case report and Chinese literature review reported in China mainland

Nasal natural killer (NK)/T cell lymphoma is an Epstein-Barr virus (EBV) associated lymphoma that arises in the nasal area and aggressively invades surrounding tissues. Our patient was a 48-year-old male who had had nasal obstruction and nasal discharge for 2 years and infiltrating plaques and necrosis on his nasal dorsum for three months. He developed fever and fatigue two weeks before admission. Biopsy from both skin and nasal mucosa revealed atypical medium-sized tumor cells infiltrating into the dermis. Immunohistochemical studies revealed that the tumor cells were UCHL-1, cytoplasmic CD3, CD56, TIA-1, and granzyme B positive, and CD8 and CD20 negative. In situ hybridization for EBV-DNA was positive. Clonal TCRb and TCRg gene rearrangement were negative. The patient was treated with cyclophosphamide, vincristine, and prednisone (COP) and with local radiotherapy, but he died 20 days later. We reviewed the cases of nasal NK/T cell lymphoma reported in mainland China in the Chinese literature during the last 5 years.     

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.     

Primary cutaneous CD20-positive T-cell lymphoma

CD20 is a transmembrane protein that is expressed by B cells during their development and is, therefore, commonly used to label cells of B lineage in lymphoid infiltrates. CD20-positive T-cell lymphoma is infrequent but well recognized. Cases reported in the literature show a variety of clinical and histoimmunochemical profiles. Primary cutaneous CD20-positive T-cell lymphoma is vanishingly rare; only eight cases have been previously reported. We present two new cases of this entity and describe their clinical, histological and immunohistochemical features. CD20 is a highly specific B-cell marker. However, it has been reported in a subset of normal T-cells in peripheral blood and bone marrow of healthy individuals. This subset of T-cells also expresses more often CD8 and g/d than the CD20-negative T-cells. Two main theories have been postulated to explain the expression of CD20 by neoplastic T-cells. The first possibility is that these lymphomas develop from the CD20-positive subset of normal T-cells. The second theory regards CD20 as an activation marker. Prognostic implications and therapeutic options of T-cell lymphomas with positivity for CD20 remain to be elucidated.     

Primary cutaneous marginal zone lymphoma with subclinical cutaneous involvement and biclonality

Primary cutaneous extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) represents a monoclonal B-cell neoplasm that typically presents with papules, plaques or nodules. We describe a patient with a primary cutaneous MALT lymphoma with unusual clinical features and an unusual immunophenotype. Conventional microscopy together with immunohistochemistry and in-situ hybridization showed the presence of lymphoma in normal-appearing and minimally erythematous skin as well as in clinically involved skin. Furthermore, at least two distinct clones were shown, one of which had κ-light chain restriction, and the other of which had λ-light chain restriction. This case represents a newly described clinical appearance of primary cutaneous MZL and shows that some patients may have more than one neoplastic clone.