皮肤原发性侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤一例

患者女,19岁。全身反复发作进行性溃疡伴发热1年。皮损初起为紫红色斑丘疹,迅速破溃形成溃疡,伴剧烈疼痛。皮损渐发展至躯干、四肢,伴间歇性高热。皮肤组织病理示真皮全层及皮下脂肪层结节状中至大异形淋巴样细胞浸润,伴局灶性亲表皮生长。浸润细胞免疫组化标记CD3、CD8、T细胞细胞内抗原、T细胞受体β均阳性。T细胞受体基因重排提示T细胞克隆性增生,确诊为皮肤原发性侵袭性亲表皮CD8阳性细胞毒T细胞淋巴瘤。患者通过环磷酰胺、长春新碱、泼尼松、博来霉素化疗,皮损部分好转,但仍于发病后22个月死亡。     

Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma

An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the DNA extracted from the specimen. A diagnosis of CD8+ cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8+ primary CTCL with clinical features resembling HV.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤1例临床病理观察并文献复习

报告1例原发性皮肤CD4 多形性小/中T细胞淋巴瘤.患者女,45岁.右膝右上方反复红斑、结节15年.组织病理检查示真皮全层及皮下脂肪层弥漫性结节性致密小到中等大淋巴样细胞浸润.细胞有异形,其间混杂少量炎性细胞,无亲表皮现象.免疫组化检查示全T抗原缺失的Th表型.诊断:原发性皮肤CD4 多形性小/中T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 aggressive epidermotropic CD8(+) T-cell lymphoma: Proposed diagnostic criteria and therapeutic evaluation

Primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma is a rare cytotoxic lymphoma characterized clinically by aggressive behavior and histologically by prominent epidermotropism of atypical CD8(+) lymphocytes. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is still awaiting. Herein, we study and analyze the different clinical, histopathological, and immunohistochemical features described in the reported cases. Different therapeutic modalities and their impact on the prognosis of the tumor are also evaluated and presented. We propose two sets of diagnostic criteria. The first comprises constant clinical, histopathological, and immunohistochemical features that are always present in every case, and the combination of which is necessary for the diagnosis. The second set helps to avoid missing cases and includes variable features that may be present in some cases, and to which any emerging finding could be added. Although different therapeutic options have been used, either as single agents or in combinations, there is no standard therapy for primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma and the tumor still represents a therapeutic challenge with very poor prognosis.     

Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma with aggressive course

BACKGROUND: Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the literature. Below we report a case involving unusual association of cutaneous vasculitis and lymphoproliferation. CASE REPORT: A 42-year-old senegalese man was hospitalized for cutaneous nodular lesions, which rapidly spread and became necrotic and ulcerated. he had recent weight loss with fever and multiple enlarged lymph nodes. Cutaneous histological analysis showed epidermotropic dermal infiltrate comprising medium and large cd8+ cytotoxic t-cells of unusual angiocentricity with cutaneous vasculitis and fibrinoid necrosis. the patient died 4 months after initiation of treatment with multi-agent chemotherapy. DISCUSSION: This patient presented the characteristics of primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma described by Berti. The clinical findings in most cases consist of nodular and ulcerative cutaneous lesions. Histologically, the cutaneous infiltrate is composed of pleomorphic lymphocytes with marked and constant epidermotropism. Immunohistochemistry shows lymphocytes expressing a CD8+ phenotype and cytotoxic proteins, which probably accounts for the local and systemic aggressiveness of the disease, as well as the angiodestructive nature of the infiltrate and the necrotic lesions.     

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with a CD15(+)CD30(-) phenotype

Primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma (CD8(+)TCL) is an extremely rare entity with distinct clinicopathological features. While the CD15 antigen is typically associated with classic Hodgkin's lymphoma, aggressive peripheral T-cell lymphomas, including advanced stage cutaneous T-cell lymphomas, rarely express this molecule. We report a case of primary cutaneous aggressive epidermotropic CD8(+)TCL, in which lymphoma cells are CD15(+)CD30(-) with a medium-to-large pleomorphic phenotype. Although the functional characteristics of CD15 expression in the cutaneous lymphomas are not fully understood, the poor prognosis of primary cutaneous aggressive epidermotropic CD8(+)TCL might be associated with the presence of this molecule in our case.     

足部皮肤T细胞淋巴瘤1例

报告1例足部皮肤T细胞淋巴瘤。患者男,63岁。左足跟部肿胀4个月,抗感染治疗无效,皮肤组织病理和免疫组化均符合T细胞淋巴瘤。     

皮肤CD8+亲表皮性T细胞淋巴瘤一例

75岁男性患者,躯干、四肢出现瘙痒性红斑、斑块和苔藓样皮疹5年.皮肤科检查:颈部、躯干和四肢泛发暗红、灰黑色浸润性斑片和斑块,以背部为甚.全身浅表淋巴结无肿大,未发现系统受累情况.组织病理显示:表皮不规则增生,棘层肥厚,表皮内可见核深染的异形淋巴细胞浸润,形成Pautrier微脓肿.真皮浅层有片状和团块状淋巴样细胞浸润,细胞有异形性.免疫组化标记:肿瘤细胞CD3、CD8、GrB、LCA和TIA-1阳性,CD4和CD5阴性.符合原发性皮肤CD8+亲表皮性T细胞淋巴瘤诊断.口服维胺酯治疗病情有所好转.     

Erythrodermic syringotropic cutaneous T-cell lymphoma

Syringotropic cutaneous T-cell lymphoma (CTCL) is a rare localized variant of CTCL, characterized histologically by eccrine gland and ductal hyperplasia surrounded by a dense syringotropic lymphocytic infiltrate. Previously reported only in men, we describe the first woman with syringotropic CTCL. Unusually, she presented with erythroderma, cutaneous nodules, poikilodermatous patches, widespread alopecia and lymphadenopathy.     

Phototherapy for cutaneous T-cell lymphoma

Phototherapy has been utilized for decades in the treatment of various dermatologic conditions, including cutaneous T-cell lymphoma (CTCL). Currently, a number of light sources are available, and selection of the specific modality is based on a number of factors, the most important of which is disease stage. The efficacy of broadband ultraviolet B (UVB) is limited to the patch stage, while psoralen and ultraviolet A (PUVA) is capable of clearing plaques and, sometimes, early tumors. Narrowband UVB is also effective for early stages and has practical advantages over PUVA, but more studies are needed to more fully evaluate its role in CTCL. Long-wave ultraviolet A (UVA1) has likewise shown efficacy, supported by findings of apoptosis induction in UVA1-treated cells. Long-term remissions have been reported for PUVA, but in the majority of cases, maintenance therapy was necessary. Although beneficial as monotherapy for early stages of the disease, phototherapy is also a useful adjunct to other modalities such as interferons, retinoids and electron beam therapy. Studies are ongoing to refine protocols for combination therapy, with the goal of improving efficacy, while minimizing adverse effects.     

Ofuji papuloerythroderma evolving to cutaneous T-cell lymphoma

Ofuji papuloerythroderma is an uncommon entity of unknown aetiology, characterized by a pruritic eruption of widespread, red–brown, flat papules that leads to spare skin folds. A number of cases have been described associated with tumour pathology, mainly cutaneous T‐cell lymphomas. We report a new case of Ofuji papuloerythroderma evolving to cutaneous T‐cell lymphoma in an 85‐year‐old woman who had been previously diagnosed with papuloerythroderma 7 years previously.     

CD30-positive cutaneous T-cell lymphoma with concurrent solid tumour

Extranodal CD30+ T-cell lymphomas seldom carry classical t(2;5) translocation and are usually anaplastic large cell lymphoma kinase protein negative. They cover a wide spectrum of histological and clinical behaviour. The prognosis of CD30+ cutaneous T-cell lymphoma (CTCL) is good in the absence of nodal primary or disseminated disease. These lesions can undergo spontaneous regression, and overlap with the group of lesions of lymphomatoid papulosis. Although an increased incidence of solid tumours has been reported in patients with CD30+ non-Hodgkin lymphoma of the skin, reports of concurrent malignancies are rare in CD30+ CTCL. We report two patients with CD30+ CTCL who, respectively, had concurrent disseminated gastric carcinoma and bilateral ovarian teratoma. Despite an aggressive clinical and histological appearance, both cases ran favourable clinical courses. The CTCL responded completely to chemotherapy in one patient, who eventually succumbed to gastric cancer. In the other patient, lesions regressed spontaneously after bilateral oophorectomy. A possible relationship between the lymphoma and the solid tumours is discussed.     

Fatal CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma with multiorgan involvement

A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient's history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago.At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+ extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial workup. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, the CD8+ epidermotropic cytotoxic subset comprises rare, highly aggressive forms characterized by metastatic spread to unusual sites such as the oral cavity, lungs, testis and the central nervous system but usually not to the lymph nodes. These cases seem to be distinct from mycosis fungoides with CD8+ phenotype, which shows a nonaggressive clinical behavior.     

Treatment planning in cutaneous T-cell lymphoma

Effective long-term management of cutaneous T-cell lymphoma (CTCL) requires administration of skin-directed therapies such as topically applied nitrogen mustard or photochemotherapy to achieve a complete response in clinically early disease (patch and thin-plaque-phase mycosis fungoides, MF) and often the concomitant administration of well-tolerated drugs with systemic effects such as interferon alfa, bexarotene, methotrexate or extracorporeal photopheresis in more advanced, but not highly aggressive/nontransformed disease (thick plaque or tumor phase MF or erythrodermic CTCL). The author's approach is provided as a guide for dermatologists in private practice.     

Erythema multiforme‐like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T‐cell lymphoma: A diagnostic and therapeutic challenge

Primary cutaneous aggressive cytotoxic epidermotropic CD8+ T‐cell lymphoma is an extremely rare, rapidly progressing, cutaneous lymphoma, with frequent systemic involvement and poor prognosis, that still represents a diagnostic and therapeutic challenge, especially in the early stage. Herein, we report a case of an elderly woman with a fulminant course, who at onset presented with clinical and pathological features mimicking erythema multiforme (EM) and treated with cyclosporine that led to rapid deterioration with fatal outcome 6 months after disease onset. Histopathology showed a lichenoid, epidermotropic and nodular, angiocentric, dermal and subcutaneous infiltrate of sF1, CD8+, CD45RA+ small to medium‐sized atypical lymphoid cells, which strongly expressed cytotoxic markers. Monoclonal T‐cell‐γ receptor was clonally rearranged and array‐CGH showed numerous chromosomal imbalances. This case evidences the clinical, pathological and therapeutic challenges involved in this tumor. The first biopsy showed an interface dermatitis‐like pattern, revealing the deceptive features that early cutaneous infiltrates of this aggressive lymphoma may have. A high suspicion for aggressive CTCL and a low threshold for repeat biopsies should be maintained when faced with rapidly progressing and/or ulcerative EM‐like lesions, especially if immunomodulatory therapy is being considered.