早期呈丹毒样损害的原发性皮肤弥漫大B细胞淋巴瘤

患者男,69岁。右下肢红肿3个月,结节1个月余。患者早期右下肢皮肤红肿呈丹毒样损害,抗感染治疗欠佳,病程进展出现肿胀加重及多个紫红色结节。皮肤科检查:右下肢肿胀,可见多个紫红色结节,部分融合。右大腿及颈部散在豌豆大的红色或肤色结节。皮损组织病理检查:表、真皮可见无浸润带,真皮内及皮下脂肪层可见弥漫淋巴样细胞浸润,淋巴样细胞核大、深染,异形性明显,部分呈泡状核,有单个中位核仁,可见核分裂象。免疫组化:肿瘤细胞表达CD20、CD10、转录因子(MUM)-1、B细胞淋巴瘤/白血病(Bcl)-2;灶状表达Bcl-6;不表达CD3、CD5、CyclinD1及CD30;原癌基因(Cmyc)约70%阳性;核增殖抗原(Ki-67):阳性细胞约90%。诊断:原发皮肤弥漫大B细胞淋巴瘤(腿型)ⅢA期。予化疗后,皮损明显好转。     

婴儿头皮前B淋巴母细胞性淋巴瘤

报道1例婴儿头皮前B淋巴母细胞性淋巴瘤。患儿男,9个月,因头皮肿块,脱发6个月余就诊。皮损表现为7．5cm×10．5cm浸润性肿块,呈暗红色,形态不规则,皮损区头发稀疏伴斑状脱发。皮肤活检示真皮及皮下组织弥漫性分布肿瘤细胞,瘤细胞中等大小,胞质少,核呈圆或卵圆形,核仁小,可见核分裂相,表真皮之间有细胞浸润带将表真皮分开。免疫组化CD20、BCL-2、Ki-67和PAX5均呈阳性表达,CD3、TdT、CD30、AI。K、CK5／6、CD56、TIA-1、CD43、CD117、穿孔素和颗粒酶B为阴性。     

原发皮肤的母细胞性浆细胞样树突细胞肿瘤二例

目的 报道2例母细胞性浆细胞样树突细胞肿瘤(BPDCN).方法 从临床表现和组织病理学、免疫表型、治疗及预后对2例BPDCN进行分析.结果 2例患者均为女性,皮损为首发症状.例1结节位于腰背部及左上臂,呈紫红色,组织病理:真皮及皮下脂肪层大量淋巴样细胞呈弥漫性浸润,浸润区与表皮之间形成无浸润带.例2皮损为左膝关节皮下肿块,组织病理:真皮及皮下组织淋巴样细胞呈结节状浸润.2例免疫组化均示CD4、CD56、CD123强阳性,Ki-67阳性约40％,MPO、EBER阴性.例1 TdT阴性,例2 TdT阳性.骨髓象均显示:粒系、红系及巨核系增生活跃,分叶阶段细胞比例增高.骨髓免疫表型均显示:未累及骨髓.血常规均示贫血.例1于确诊2个月后死亡,例2经VDLP及CHOP化疗后皮损消退,并进行同种异体骨髓移植,目前状况良好,皮损无复发.结论 BPDCN是少见的常以皮损为首发症状的恶性肿瘤,以迅速生长和高度侵袭性为特征,预后不良,无标准的治疗方法,早期诊断,进行治疗干预可改善患者预后,异体骨髓移植可明显延长生存期.     

非霍奇金淋巴瘤1例

报告1例以皮肤肿瘤为首发表现的儿童非霍奇金淋巴瘤。患儿女,8岁。右侧鼻翼出现肿块3个月余,伴进行性增大1个月就诊。体格检查示局部淋巴结不增大,系统检查无异常。皮损组织病理检查示真皮内有异形淋巴样细胞浸润,免疫组化染色结果示：CD45RO（＋）,CD20,HMB45,CK,CD30和CD68均阴性,证实为T细胞淋巴瘤。     

肉芽肿性蕈样肉芽肿二例并文献复习

报道2例以局部浸润性斑块为主要表现的肉芽肿性蕈样肉芽肿并复习相关文献.2例患者均表现为红斑、斑块,皮损组织病理检查见真皮内淋巴样细胞及巨细胞浸润,患者1免疫组化示CD2、CD3、CD5、CD7阳性,患者2 CD2、CD3、CD4阳性及CD68组织细胞阳性.诊断:肉芽肿性蕈样肉芽肿.患者1口服阿维A及肿块局部浅层X线照射...     

急性髓细胞性白血病(M4型)

报告1例急性髓细胞性皮肤白血病(M4型).患者女,48岁.全身出现丘疹、红色结节14d,伴剧烈瘙痒.体格检查:全身泛发大小不等的红色丘疹、结节,质韧,无压痛.皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,有明显异形及较多核分裂象.免疫组化组织病理检查:CD68阳性(灶性),MPO阳性(少量).骨髓穿刺:白血病细胞大量增生,免疫标记:CD68、CD11b、MPO及HLA-DR均阳性.诊断:急性髓细胞性白血病(M4型).患者经过2次DA(伊达比星、阿糖胞苷)方案化疗后,再次行骨髓穿刺示缓解,但皮损仍有复发.     

肉芽肿性皮肤松弛症

报告1例肉芽肿性皮肤松弛症。患者女,46岁。双侧腹股沟红斑、条索状肿块伴疼痛1年。皮肤科检查:双侧腹股沟浸润性红斑,左侧腹股沟可触及4 cm×10 cm条索状质硬肿块,表面皮肤松弛。皮损组织病理检查:真皮内大量淋巴样细胞浸润,可见淋巴细胞亲表皮现象,真皮内可见散在多核巨细胞。免疫病理示淋巴样细胞CD2、CD5及CD4均阳性;多核巨细胞CD68/PGM-1阳性。弹性纤维染色可见部分区域弹性纤维减少。TCR-γ基因重排示克隆性扩增峰。诊断:肉芽肿性皮肤松弛症。     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

Atrophoderma of moulin with preceding inflammation

A 16-year-old Vietnamese man presented to the Dermatology Clinic with a 10-year history of bizarre brown patches, which initially started as red asymptomatic "bumps" on the trunk, upper and lower extremities, and face. His past medical history was significant for hypothyroidism and idiopathic urticaria. He was on Eltroxin for hypothyroidism. The family history was noncontributory. Physical examination revealed two types of lesion: erythematous, well-circumscribed papules in a linear configuration along with linear hyperpigmented atrophic patches following Blaschko's lines were noted on the lower extremities (Fig. 1), right upper extremity, right flank (Fig. 2), and right jawline. Initial biopsies taken from the papular lesions on the right thigh and right elbow revealed the following changes. The first biopsy showed a slightly thinned epidermis with prominent dilated blood vessels in the superficial dermis. There also appeared to be a slight increase in the amount of collagen in the deep dermis. The findings were reported as in keeping with "epithelial atrophy." The second biopsy from the lesion on the right elbow revealed an acanthotic epidermis. The granular layer was absent in several areas and there was marked overlying parakeratosis. In the dermis, there was a heavy perivascular lymphocytic infiltrate. The appearances were consistent with a psoriasiform dermatitis (Fig. 3). A biopsy taken from the left thigh approximately 18 months later showed slight irregular acanthosis with dermal edema, dilated blood vessels, and a patchy lymphocytic infiltrate. The appearances were compatible with mild inflammation.     

急性髓细胞性白血病M2型所致皮肤白血病一例

报道1 例急性髓细胞性皮肤白血病.患者男,38岁,因确诊急性粒细胞性白血病1年,躯干部皮疹1个月入院.皮肤科检查:躯干、右额部皮肤散在数十个直径约1～2 cm大小的红色结节及斑块,略高出皮面,触之有明显浸润感,无压痛.骨髓检查及免疫学证实为急性髓细胞性白血病M2型,患病1年后躯干及前额皮肤出现斑块及结节,皮肤组织病理及免疫组化证实为皮肤白血病.白血病短期内出现皮肤、肺、脾、肾、胸腰骶椎多发性浸润,则提示病情急性进展及预后不良.     

Focal dermal hypoplasia (Goltz syndrome) associated with multiple giant papillomas

We report a case of focal dermal hypoplasia (FDH) with multiple giant papillomas on nonperimucosal areas. The patient had had cribriform hyperpigmented and depigmented plaques on the trunk and extremities since birth. There were also hypoplastic skin lesions on the right arm, left elbow and right thigh. The multiple giant papillomas began to appear. when she was 22 years old, on the trunk and extremities. Cryotherapy was effective in controlling them.     

Clinicopathologic correlations in leukemia cutis

This clinicopathologic study involved 42 cases of leukemia cutis: 3 of acute lymphocytic leukemia (ALL), 16 of chronic lymphocytic leukemia (CLL), 12 of acute granulocytic leukemia (AGL), 3 of chronic granulocytic leukemia (CGL), 5 of acute monocytic leukemia (AML), and 3 of acute myelomonocytic leukemia (AMML). The clinical appearance of leukemia cutis included papules, macules, plaques, nodules, ecchymoses, palpable purpura, and ulcerative lesions, and these were seen in all types of leukemias. Gingival hypertrophy was seen only in AML or AMML, and erythroderma and bullous lesions of leukemic infiltration were observed only in CLL. Cutaneous leukemic lesions may be concomitant with or preceding the diagnosis of systemic leukemia. Therefore, skin biopsy may be helpful in detecting the leukemia and may facilitate the work-up. Leukemia cutis probably is a dissemination of systemic leukemia to the skin, and the demonstration of leukemia in skin is associated with a very poor prognosis.     

过敏性紫癜合并急性播散性脑脊髓炎

报告1例过敏性紫癜并发急性播散性脑脊髓炎。患者女,31岁。躯干及四肢反复瘀点和瘀斑2余年。体格检查:双侧眼球外展受限,颈软,肌力和肌张力均正常,脑膜刺激征(-),双侧Chaddock征(+),右侧抓握反射(+)。皮肤科检查:四肢、腹部、腰部及背部多发对称性分布的紫红色斑疹及斑丘疹,部分融合成片,皮损压之不褪色。皮损组织病理检查:表皮大致正常;真皮浅中层细血管周围及胶原束间小片状淋巴细胞浸润,伴稍多中性粒细胞及核尘,部分血管管壁模糊,可见红细胞漏出。头颅磁共振成像(MRI)示两侧大脑半球、基底节区及右侧桥臂多发脱髓鞘病变。诊断:过敏性紫癜合并急性播散性脑脊髓炎。     

Linear IgA bullous dermatosis in a patient with acute lymphocytic leukemia: possible involvement of granulocyte colony-stimulating factor

We describe a case of linear IgA bullous dermatosis (LABD) in a patient with acute lymphocytic leukemia during treatment with granulocyte colony-stimulating factor (G-CSF). After a drug eruption due to imipenem cilastatin sodium had disappeared, bullous lesions appeared on the trunk. Results of histopathological studies and direct immunofluorescence studies of the lesion were consistent with LABD. Reinstitution of G-CSF after the resolution, however, did not reproduce the bullous eruptions. This suggests that in addition to G-CSF, the presence of precipitating factors that can synergistically enhance or accelerate the outbreak of the disease is required for the development of bullous lesions. Various cytokines, such as interleukin-2 (IL-2) and interferon-gamma (IFN-gamma), endogenously produced from activated lymphocytes during the drug eruption might have provided a favorable milieu for the onset of G-CSF-induced LABD. We suggest that patients with LABD will need special attention with respect to the type of cytokines or combination of cytokines given as therapeutic modalities.     

伴有皮肤表现的慢性B淋巴细胞白血病

报告1例伴有特异性和非特异性皮损的慢性B淋巴细胞白血病.患者男,66岁.临床表现为全身广泛淋巴结增大;躯干及四肢出现红斑、丘疹、坏死;左耳郭有浸润性肿块.皮损组织病理检查示左耳郭皮损为淋巴细胞白血病皮肤浸润,其余皮损为血管周围炎及脂膜炎改变.经淋巴结组织病理、骨髓细胞学、骨髓组织病理及流式细胞仪检测诊断为慢性B淋巴细胞白血病.     

The spectrum of cutaneous disease in leukemias

Cutaneous eruptions are frequent complications in the clinical course of patients with leukemia. Leukemia cutis is occasionally the cause of the eruption, but in many cases the lesions are non-leukemic. We have retrospectively selected all skin biopsies from patients with a computer-coded diagnosis of leukemia seen in the Stanford University Department of Pathology in the last 7 years, and separated these cases into the broad categories of acute myelogenous leukemia (AML), acute lymphocytic leukemia (ALL), chronic myelogenous leukemia (CML), and chronic lymphocytic leukemia (CLL). We also analyzed separately those cases seen in patients treated with bone marrow transplantation from those treated with standard chemotherapy regimens. We found that leukemia cutis was seen frequently as the cause of lesions in patients with CML and CLL. In contrast, a wide variety of lesions were seen in patients with AML, including a greater number of infectious lesions, drug reactions, vasculitis, and lesions secondary to a hemorrhagic diathesis. In the bone marrow transplantation patients, graft vs host disease was usually the cause of skin lesions in those transplanted for CML and ALL, but again those patients with an underlying diagnosis of AML showed a wide variety of lesions including drug reactions, fungal infections and leukemia cutis. Finally, 6% of cases from patients with AML showed intraepidermal blistering disorders of various types, an association that has not been previously reported.     

Insect bite-like reaction in patients with hematologic malignant neoplasms

BACKGROUND: Exaggerated reaction to insect bites, mainly to mosquitoes, is infrequently described in patients with chronic lymphocytic leukemia. Skin lesions usually appear months to years after the diagnosis of leukemia and are unrelated to laboratory findings, disease course, or therapy. OBSERVATIONS: We describe 8 patients with various hematologic disorders (chronic lymphocytic leukemia, acute lymphoblastic leukemia, acute monocytic leukemia, mantle-cell lymphoma, large-cell lymphoma, and myelofibrosis) who developed insect bite-like reaction. Although the clinical picture and the histological characteristics of the lesions were typical for insect bites, none of the patients actually had a history, course, or response to treatment suggestive of arthropod assaults. In 2 patients, the eruption preceded the diagnosis of the malignant neoplasm. The rash persisted for months to years and was resistant to therapies other than systemic corticosteroids. The 3 patients with chronic lymphocytic leukemia seemed to have a worse prognosis than expected for their disease. In 1, the polymerase chain reaction detected leukemic cells in the infiltrate. CONCLUSIONS: Insect bite-like reaction is an infrequent, disturbing, and difficult-to-treat nonspecific phenomenon in patients with hematologic malignant neoplasms. Since it may precede the hematologic disorder, oriented evaluation is warranted. We speculate that immunodeficiency plays a role in its pathogenesis; however, the exact pathogenesis and its prognostic implications await further studies.     

Concurrent eosinophilic fasciitis and cutaneous T-cell lymphoma. Eosinophilic fasciitis as a paraneoplastic syndrome of T-cell malignant neoplasms?

Eosinophilic fasciitis has been reported to precede hematologic malignant neoplasms such as myelomonocytic leukemia, lymphocytic leukemia, and Hodgkin's lymphoma. In this case study, eosinophilic fasciitis occurred concurrently with cutaneous T-cell lymphoma (mycosis fungoides). The clinical diagnosis of eosinophilic fasciitis was based on painful sclerodermatous lesions on the extremities and trunk without acrosclerosis. There was histologic confirmation with edema and lymphocytic inflammation in the superficial muscular fascia and dermis. Deposition of immune reactants was found in the fascia and dermis. In addition, peripheral eosinophilia and circulating immune complexes were detected. The diagnosis of cutaneous T-cell lymphoma (mycosis fungoides) was based on extensive erythematous cutaneous plaques, dermal and epidermal lymphocytic atypia, loss of pan-T-cell immunologic markers, and a cutaneous lesional T-cell receptor beta-chain rearrangement by Southern blot analysis. Eosinophilic fasciitis may occur as a paraneoplastic syndrome associated with hematologic malignant neoplasms, including mycosis fungoides. Cytokines or lymphokines released by activated immunocytes, either malignant leukocytes or normal leukocytes reacting to malignant cells, may be responsible for the eosinophilia and sclerosis seen in these associated hematologic malignant neoplasms.