Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with atypical presentation

Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma is characterized by a proliferation of epidermotropic CD8(+) cytotoxic T cells and an aggressive clinical behavior. Patients present with localized or disseminated eruptive papules, nodules and tumors. We report a case of primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with unusual clinical manifestation. The lesion occurred as multiple brownish macules and flat-topped papules on the hands, feet and face in a 25-year-old woman.     

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.     

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.     

Cytotoxic/suppressor (CD8+, CD4-) cutaneous T-cell lymphoma with aggressive course

A case of cutaneous cytotoxic/suppressor T-cell (CD8+, CD4-) lymphoma is reported. The tumor was characterized by its rapid growth and no response to polychemotherapy. This unusual immunophenotype seems to be associated, in this and other cases reported previously, with a more aggressive course than the classic indolent course of cutaneous T-cell lymphoma.     

Primary cutaneous CD30+ T-cell lymphoma responsive to topical imiquimod (Aldara)

CD30+ anaplastic large cell lymphoma is a primary cutaneous lymphoproliferative disorder with a high rate of spontaneous regression (almost 25%). The suggested therapies are radiation, surgery and methotrexate. We describe two patients with nonregressing primary cutaneous CD30+ T-cell lymphoma that was successfully treated with topical imiquimod 5% cream (Aldara, 3M) three times weekly for 6 weeks. In both cases we obtained complete clinical remission, confirmed by histology. No recurrences were observed during the following 8 months. We consider that topical application of an immune response modifier, such as imiquimod, could be a good alternative to other potentially more dangerous or aggressive treatments.     

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.     

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。     

Progressive epidermotropic CD8+/CD4- primary cutaneous CD30+ lymphoproliferative disorder in a patient with sarcoidosis

We describe a patient with a CD8+/CD4- primary cutaneous CD30(+) lymphoproliferative disorder with striking epidermotropic histology and coincident cutaneous and systemic sarcoidosis. This patient illustrates the spectrum of clinical and histologic features of CD30+ lymphoproliferative disorders and the need for adequate staging in such cases. This patient's CD30/CD8 coexpression is rare and has clinical and prognostic implications, including mucosally and acrally accentuated lesions and a potentially more aggressive course. Primary cutaneous CD30+ lymphoproliferative disorders have an excellent prognosis; therefore multiagent chemotherapy modalities are generally not indicated. The combination of T-cell lymphoma and sarcoidosis is also rare and may limit treatment options.     

Primary cutaneous CD30+ large T-cell lymphoma in a patient with psoriasis treated with cyclosporine

We report the case of a 61-year-old woman who developed an anaplastic CD30+ cutaneous T-cell lymphoma during oral cyclosporine (CsA) therapy for recalcitrant psoriasis. Two months after CsA discontinuation, clinical and histological resolution of the lymphoma was observed. However, 3 years later extracutaneous involvement of the lymphoma could be detected. The association between CsA administration and the occurrence of the lymphoma may be casual, but a relationship with immunosuppression may also be hypothesized. We have reviewed all relevant data in the literature. To our knowledge, this is the first case of primary cutaneous CD30+ anaplastic large T-cell lymphoma in a patient treated with CsA for psoriasis.