Cutaneous presentation of recurrence of lymphoepithelioid T-cell lymphoma (Lennert's lymphoma)

Lennert's lymphoma (LL) is a T-cell lymphoma characterized by the presence of atypical T lymphocytes, admixed with histiocytes and epithelioid granulomas. Patients present with superficial lymph node involvement, mainly in the cervical areas; thoracic adenopathies and involvement of deep abdominal lymph nodes are rare. Cutaneous involvement is infrequent, reported to occur in only 4-11% of patients, and even rarer is the onset of cutaneous lesions as first sign of a recurrence. We report a female patient who presented with papules and nodules on the trunk and upper limbs as the first manifestation of recurrent LL.     

Cutaneous lymphoepithelioid lymphoma (Lennert's lymphoma). Combined immunohistological, ultrastructural, and DNA-flow-cytometric analysis

Lymphoepithelioid lymphoma (LEL) is a peculiar type of low grade malignant T cell lymphoma usually arising in lymph nodes, characterized immunohistologically by predominant T-helper/inducer lymphocytes intermingled with clusters of epithelioid cells. An uncommon case of LEL with cutaneous involvement is reported with additional reference to ultrastructure and DNA-flow-cytometric analysis (DNA-FCM) of the lymphoma. Light microscopy showed subepidermal bandlike infiltrates of lymphocytes and clusters of epithelioid cells extending into the subcutaneous tissue. By immunohistochemistry the presence of a high percentage of T-helper/inducer lymphocytes was confirmed. DNA-FCM demonstrated an aneuploid cell population indicating malignant cells. Our results are in accordance with earlier ones established in lymph nodes.     

Cutaneous T-cell lymphoma presenting as papuloerythroderma—a case and review of the literature

Papuloerythroderma has been described as a distinct clinical entity. It is characterized by pruritus, red flat-topped papules with skin crease sparing, blood eosinophilia and lymphopenia. It has been described almost exclusively in elderly men. We report a case of cutaneous T-cell lymphoma which, at presentation, was indistinguishable from papuloerythroderma, in a 79-year-old male. An excellent clinical response to low dose oral corticosteroids and photochemotherapy was observed. There have only been two previous reports linking papuloerythroderma with T-cell lymphoma. This patient is in keeping with the two previously reported cases and would support the view that papuloerythroderma may predispose to, or in fact represent, an early form of cutaneous T-cell lymphoma.     

Primary cutaneous peripheral T-cell lymphoma,unspecified with an indolent clinical course: a distinct peripheral T-cell lymphoma?

Primary cutaneous peripheral T-cell lymphomas (PTL), unspecified, are rare lymphomas, with a poor prognosis. They grow and disseminate rapidly, leading to widespread disease. We report a case of PTL, unspecified occurring on the nose. Despite its aggressive histology, this tumour behaved indolently. It is remarkably similar, clinically and histologically, to four recently described cases that occurred on the ear.     

Cutaneous Hodgkin's lymphoma: a case report and review of the literature

报告1例皮肤霍奇金淋巴瘤.患者男,28岁.因右侧锁骨上不规则鲜红色斑块1年就诊,全身系统检查未见肿大淋巴结和内脏肿瘤,经皮损组织病理和免疫组化检查诊断为皮肤霍奇金淋巴瘤.     

皮肤母细胞性NK细胞淋巴瘤一例

患者男,20岁,因右大腿起皮疹5个月,泛发全身2个月余就诊.体检:躯干、四肢散在分布的蚕豆至鸽蛋大小的暗红色结节,表面无破溃,质韧,无压痛.全身浅表淋巴结未扪及.背部皮损组织病理及免疫组化染色证实为母细胞性NK细胞淋巴瘤.经依托泊苷+环磷酰胺+长春新碱+地塞米松+甲氨蝶呤方案化疗后缓解.     

Cutaneous malakoplakia. Report of a case and review of the literature

Cutaneous malakoplakia occurring in the perianal region of a 64-year-old man is reported and the features by light and electron microscopy are described. Review of the literature disclosed that cutaneous malakoplakia is rare, there having been only seven previous cases reported. Current concepts on the pathogenesis of this condition are discussed.     

Cutaneous manifestations in the cardiofaciocutaneous syndrome, a variant of the classical Noonan syndrome. Report of a case and review of the literature

We report one patient with cardiofaciocutaneous (CFC) syndrome. He presented with clinical findings characteristic of this condition such as: cutaneous abnormalities, including ichthyosis, widespread keratosis pilaris, a peculiar craniofacial appearance with sparse, curly hair and low-set posteriorly rotated ears; congenital heart defects; and mild mental and motor retardation. We submit a comprehensive review of previously published articles regarding the dermatological findings in CFC syndrome (recently shown to be a variant of Noonan syndrome) emphasizing diagnostic criteria and its differentiation from the Costello syndrome.     

Acrokeratosis paraneoplastica (Bazex' syndrome). Report of a case and review of the literature

Acrokeratosis paraneoplastica (Bazex' syndrome) is a rare but clinically distinctive dermatosis that has been associated in all reported cases, to our knowledge, with either a primary malignant neoplasm of the upper aerodigestive tract or metastatic cancer to the lymph nodes of the neck. Acrokeratosis paraneoplastica was found in a 53-year-old black man with squamous cell carcinoma of the tonsil. A distinctive series of changes was found on histopathologic examination of biopsy specimens taken from his skin lesions, and direct immunofluorescence microscopy of both lesional and nonlesional skin specimens showed immunoglobulin and complement deposition on the epidermal basement membrane. The skin lesions largely resolved following radiation therapy of the neoplasm and of the presumably involved lymph nodes.     

Cutaneous T-cell lymphoma and human immunodeficiency virus infection: 2 cases and a review of the literature

Cutaneous non-Hodgkin's lymphomas are rare in patients with HIV-1 infection and almost all of the cases reported are of T-cell lineage with histopathological features of mycosis fungoides or Sezary syndrome. We studied 2 cases of mycosis fungoides in HIV-1-positive patients who were intravenous drug abusers and were in stage II and IV C2 (CDC'86), respectively. The first patient (stage II) had multiple, erythematous and infiltrated large plaques on the abdomen, back, arms and legs, whereas the second patient (stage IV) had smaller erythematous, slightly scaly and infiltrated pruritic plaques on the trunk and limbs. Their CD4 lymphocyte counts were 634 and 250 cells/mm3, respectively. Biopsies showed features consistent with mycosis fungoides, with an epidermotropic pattern. The immunohistochemical study revealed a T-cell lineage of this atypical infiltrate. Both patients partially responded to topical steroid ointment, showing moderate improvement. Further biopsies performed 6 months later confirmed the prior diagnosis of mycosis fungoides. No tumour stage was observed during a 2-year follow-up. We conclude that mycosis fungoides is rare in HIV-positive patients, but must be included in the differential diagnosis of erythematous plaques in these patients. In suspected, but non-diagnostic cases of mycosis fungoides in HIV-positive patients, only a close clinical and histopathological follow-up can confirm the diagnosis.     

Cutaneous malignant mixed tumor. Report of a case and review of the literature

We describe an unusual malignant cutaneous neoplasm having a biphasic growth pattern and apparently arising in a mixed tumor. A lymph node metastasis was similar histologically to the primary lesion, exhibiting both carcinomatous differentiation and areas that appeared sarcomatous. Immunohistochemical stains showed selective staining for high molecular weight keratin in carcinomatous areas and for vimentin in areas having a sarcomatous appearance. The literature of cutaneous malignant mixed tumor is reviewed and possible explanations for the unusual morphology and immunohistochemical staining pattern are discussed.     

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.     

Intravascular cytotoxic T-cell lymphoma: A case report and review of the literature

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large B-cell lymphoma in the World Health Organization classification. Although the majority of cases are of B-cell lineage, cases of IVL with a T-cell phenotype and, rarely, histiocytic and natural killer (NK)-cell phenotypes have been reported. We report a case of T-cell IVL with a cytotoxic phenotype. A 62-year-old male presented with erythematous patches and plaques on the lower extremities, and a biopsy revealed IVL with an activated cytotoxic phenotype (CD56(+), perforin+, granzyme B+, TIA-1+, CD3epsilon(+), CD20(-), CD4(-), CD8(-), CD5(-), and T-cell receptor [TCR] betaF1(-)), consistent with either NK-cell or T-cell origin. TCR gene analysis showed a monoclonal T-cell population, supporting the diagnosis of a T-cell IVL. Although the patient's skin lesions were refractory to combination chemotherapy and salvage chemotherapy regimens, there has been no evidence of disease progression in 24 months of follow-up.     

Cutaneous involvement as the first manifestation of T‐lymphoblastic lymphoma and review of the literature

Lymphoblastic lymphomas (LBLs) are uncommon malignant neoplasms derived from immature T‐ or B‐lymphoid progenitor cells. Although cutaneous involvement may reach 33% in B‐LBL, only 12 cutaneous cases of T‐LBL have been published. We report the case of a 49‐year‐old woman with 2‐month history of erythematous‐violaceous plaques in the sternal region and breasts. Histopathologic examination showed a dense monomorphus infiltrate in dermis and positive immunostainings for CD3, CD99 and terminal deoxynucleotidyl transferase, thus indicating T‐LBL. Staging work‐up only revealed a mediastinal mass at diagnosis. After a 51‐month follow‐up and different treatment regimens, the patient remains alive although she has presented four relapses, all of them extramedullary.     

Cutaneous involvement by colonic extranodal NK/T-cell lymphoma mimicking mycosis fungoides: a case report*

We report a 51-year-old woman with cutaneous involvement by extranodal NK/T-cell lymphoma (TCL) of the colon that microscopically mimicked mycosis fungoides (MF). She had a history of fever of unknown origin for 2 months and then developed multiple erythematous papules on her trunk and extremities. A skin biopsy revealed superficial infiltration by atypical small to medium-sized lymphocytes with epidermotropism and Pautrier collections. Immunohistochemical studies showed expression of CD3 and TIA-1 with lack of expression (double negative) of CD4 and CD8. Initially, we reported the diagnosis as MF, cytotoxic variant. Thereafter, computerized tomography scan incidentally identified a colonic mass. A colonic biopsy revealed infiltration of atypical lymphoid cells with the same morphology and immunophenotype as those found in the skin. Additionally, CD56 and Epstein-Barr virus-encoded RNA in situ hybridization in both skin and colonic biopsies were diffusely positive. Thus, extranodal NK/TCL was diagnosed. Delta T-cell receptor (TCR) gene rearrangement was documented in the skin biopsy by polyacrylamide gel electrophoresis and fluorescence capillary gel electrophoresis methods. There was no TCR gene rearrangement detected in the colonic biopsy. Unfortunately, the patient died within 2 months of diagnosis.