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(+) 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.     

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.     

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.     

Aggressive T cytotoxic CD8+ epidermotropic cutaneous lymphoma: a case in a patient with Steinert's myotonic dystrophy

INTRODUCTION: Primary cutaneous "aggressive" CD8-positive epidermotropic cytotoxic T-cell lymphoma is a rare subset of cutaneous cytotoxic T/NK lymphomas that clearly differs from mycosis fungoides, whether CD4+ or CD8+, by the presence of rapidly evolving tumoral cutaneous lesions, foci of keratinocytes necrosis, a cytotoxic T phenotype and a poor prognosis. CASE REPORT: A 33-year-old man with Steinert's myotonic dystrophy was referred for evaluation of rapidly worsening cutaneous tumors along with marked deterioration of general status. Clinical, histological and immunohistological data led to the diagnosis of primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma. CHOP chemotherapy was effective despite cardiac toxicity in the setting of Steinert's dystrophy, but the patient relapsed and died of pulmonary sepsis after chemotherapy was resumed. DISCUSSION: The treatment of primary cutaneous epidermotropic CD8+ cytotoxic T-cell lymphoma is not codified. CHOP chemotherapy is usually the first-line therapy but relapses are frequent with median survival of no more than 34 months. In our patient, an additional difficulty was the cardiac toxicity of cytostatic drugs linked to the myopathy which prevented the use of high dosages, requiring a change of therapeutic regimen.     

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.     

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.     

Pleomorphic CD8+ small/medium size cutaneous T-cell lymphoma

Pleomorphic small/medium-sized cutaneous T-cell lymphoma is a recently recognized rare type of cutaneous T-cell lymphoma which is clinicopathologically different from mycosis fungoides and Sezary syndrome. By definition the phenotype of the neoplastic lymphocytes in pleomorphic small/medium-sized cutaneous CD3CD4CD8 but CD8 pleomorphic small/medium sized cutaneous T-cell lymphoma cases have been occasionally described. We describe a 55-year-old female with a pruritic erythematous nodule on the lateral aspect of her right foot present for 1.5 years. Histology revealed a nonepidermotropic lichenoid infiltrate in the papillary dermis and a patchy infiltrate in the mid and lower dermis composed of small to medium-sized pleomorphic lymphocytes. The immunophenotype of these lymphocytes was CD3CD4CD8TIA-1. Staining for CD20, CD30, CD56, TdT, and LMP1 were negative, and the Ki-67 proliferation index was 5% to 10%. Gene rearrangement studies demonstrated a T-cell clone. The laboratory and imaging workup did not reveal extracutaneous involvement. The lesion was treated by local irradiation but a follow-up biopsy demonstrated only partial remission. Consequently, the lesion was treated by surgical excision.