皮肤型Rosai-Dorfman病1例

患者男,47岁,口唇上方及左眼睑红色丘疹1个月。皮损组织病理示:真皮内可见密集的组织细胞浸润,并可见大小不等的组织细胞团块,其内可见大量的多核巨细胞,个别巨细胞内可见吞噬的淋巴细胞,团块表面混有淋巴细胞,嗜酸性粒细胞浸润。免疫组织化:S-100蛋白阳性、CD68阳性、CD1a阴性。诊断:皮肤型Rosai-Dorfman病。     

朗格汉斯细胞肉瘤

报告1例朗格汉斯细胞肉瘤。患者男,78岁。躯干、四肢红色丘疹、斑块7个月。皮疹渐增大,增多,部分融合,伴明显瘙痒。皮损组织病理检查示:表皮内及真皮浅层可见较多上皮样细胞增生,胞质丰富呈嗜酸性,细胞核大、深染,核仁明显,有明显的异形性和异常核分裂象。免疫组化结果显示肿瘤细胞S-100蛋白、CD1a和CD207阳性,CD68阴性。结合临床、组织病理及免疫组化结果诊断为朗格汉斯细胞肉瘤。     

未定类组织细胞增生症一例

76岁女性患者,全身泛发红色丘疹、结节4年。组织病理示真皮内嗜碱细胞呈结节样浸润,浸润的细胞为单一组织细胞,部分细胞核呈肾形。免疫组化提示:CD1a、S-100蛋白、CD68阳性,CD207阴性。诊断:未定类组织细胞增生症。     

窦组织细胞增生伴巨大淋巴结病

报告1例窦组织细胞增生伴巨大淋巴结病.患者男,27岁.头皮、颈部、躯干部丘疹及结节半年,伴双侧颌下淋巴结肿大3个月.皮损组织病理检查示真皮内大量组织细胞增生,可见伸入运动.淋巴结组织病理检查示淋巴窦明显扩张,窦内可见大量组织细胞及伸人运动.免疫组化染色示S-100蛋白及CD68阳性,CD1a阴性.依据临床表现、组织病理改变和免疫组化检查,确诊为窦组织细胞增生伴巨大淋巴结病.     

未定类细胞组织细胞增生症一例

报告一例未定类细胞性组织细胞增生症。患者,女,12岁。全身皮疹3+月。皮损病理检查示：表皮增生,轻度角化不全,表皮内微脓肿形成,真皮见大量体积较大,胞浆浅染,核扭曲、折叠、肾形,有核沟之细胞弥漫片状浸润,同时伴数量不等之嗜酸性粒细胞、淋巴细胞等细胞浸润。免疫组化示：CD1a(+),CD68(+),CD117(-),CD34(-),S-100(+)。电镜检查未找到Birbeck颗粒。     

间质肉芽肿性皮炎

报告1例间质肉芽肿性皮炎。患者女，17岁。因左胸部和左上肢出现呈带状分布、无自觉症状的丘疹和结节半年就诊。免疫学检查示类风湿因子阳性。组织病理检查显示真皮间质和血管周围弥漫而致密的以组织细胞为主的炎性细胞浸润，部分组织细胞核大，有异形：炎性浸润细胞中还有中性粒细胞和少量淋巴细胞、浆细胞、多核巨细胞，局部可见灶性胶原纤维变性、坏死，未见嗜酸性粒细胞浸润和血管炎改变。阿新蓝染色阴性。免疫组化染色示CD68强阳性，S-100蛋白、AE1／AE3、SMA、结蛋白、LCA、CD45RO、CD20、CD21、CD23、CD31、CD34、CD1a和Ki-67均阴性。符合间质肉芽肿性皮炎的诊断。     

表现为播散性丘疹型的皮肤型窦组织细胞增生症一例

患者男,54岁,全身红色丘疹、结节及斑块4个月。皮损初发于双下肢,渐波散至全身。查体示全身密集分布的红色丘疹、结节,孤立或呈环形、条带状分布,直径0.3~2.5 cm,质地较硬,略有压痛,有浸润感;皮损大致对称分布,以四肢伸侧、面颊部为著。组织病理示真皮内弥漫性组织细胞浸润,多个具有分枝的肥大的组织细胞及多核巨细胞浸润,灶性浆细胞、淋巴细胞、中性粒细胞浸润,少许嗜酸粒细胞浸润,有细胞伸入现象。免疫组化检查示肥大的组织细胞及多核巨细胞CD68、CD163、S-100蛋白均阳性,CD1a阴性。诊断:皮肤型窦组织细胞增多症。给予复方甘草酸苷口服及卡介菌多糖核酸肌内注射1个月,皮损部分消退。     

皮肤窦组织细胞增生症伴巨大淋巴结病1例

报告1例窦组织细胞增生症伴巨大淋巴结病。患者,男,83岁。右面部暗红色斑块、斑丘疹1年,无淋巴结增大及系统受累。皮损组织病理检查示真皮内大量组织细胞增生。免疫组化染色示S-100蛋白及CD68阳性,CD1a阴性。依据临床表现、组织病理改变和免疫组化检查,确诊为窦组织细胞增生伴巨大淋巴结病。     

误诊为系统性红斑狼疮的皮肤型Rosai-Dorfman-Destombes病一例

患者,女,60岁。面部对称性红斑1年,加重伴丘疹、斑块形成6个月。皮肤组织病理检查示：皮肤真皮深层及皮下组织弥漫性淋巴浆细胞浸润,组织细胞增生可见“伸入运动”,免疫组化S-100、CD68阳性,CD1a阴性,给予泼尼松、甲氨蝶呤、沙利度胺治疗2个月后好转。     

皮肤型Rosai-Dorfman病1例并文献复习

患者女,35岁,右前胸起红斑、丘疹、结节伴痒痛半年。皮损组织病理示:真皮可见大量密集片状、结节状分布的淋巴细胞、组织细胞、浆细胞、中性粒细胞浸润,部分组织细胞内吞噬多个形态完整淋巴细胞。免疫组织化学示:组织细胞S-100阳性、CD68阳性、CD1a阴性。诊断为皮肤型Rosai-Dorfman病,予以手术切除,随访36个月未见复发。     

S-100阳性泛发性幼年黄色肉芽肿一例并文献复习

报道1例S-100阳性泛发性幼年黄色肉芽肿患儿并对相关文献进行复习。患儿,男,6个月,面部、躯干、四肢起疹3个月余。组织病理示：表皮基本正常,真皮内可见团块状结节性浸润,浸润细胞主要为泡沫样组织细胞,梭形组织样细胞,并可见Touton巨细胞。免疫组化：泡沫样组织细胞及梭形组织样细胞CD68(+),S-100(+),CD1a(-),Langerin（-）;电镜检查示：未见Birbeck颗粒。诊断：泛发性黄色肉芽肿。     

Multiple cutaneous reticulohistiocytosis with T‐cell large granular lymphocyte clonopathy

A 63‐year‐old Caucasian man presented with a 4‐month history of disseminated asymptomatic reddish‐brown papulonodular lesions. A skin biopsy showed dermal infiltration with CD68⁺ histiocytes, predominantly with eosinophilic cytoplasm, some with a ground‐glass cytoplasm, and a small number of giant cells. The diagnosis of multiple cutaneous reticulohistiocytosis was made. Bone marrow immunophenotyping due to peripheral blood lymphocytosis revealed the presence of a monoclonal population of CD3⁺, CD8⁺ CD57⁺ large granular lymphocytes. The present case suggests the coexistence of multiple cutaneous reticulohistiocytosis with an underlying disorder.     

伴有肺部损害的多中心网状组织细胞增生症1例

报道1例伴有肺部损害的多中心网状组织细胞增生症。患者男、43岁。其临床特征为全身皮肤多发性丘疹和结节,毁损性关节炎和肺部多发性小结节。皮肤及肺结节组织病理示大量浆呈伊红和毛玻璃样外观的组织细胞和多核巨细胞浸润;免疫组化染色：CD68（＋）,S－100（－）,HHF35（－）,AE1／AE3（－）。     

POEMS syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein Skin changes

A diffuse xanthomatous infiltration was detected on a biopsy of the hyperpigmented patches in a 40-year-old man with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) syndrome. Multiple biopsies of the hyperpigmented patches showed diffuse or perivascular foamy histiocytes in the upper and mid dermis, a sparse infiltrate of lymphocytes, fibrosis, hyperpigmentation of the basal layer, and capillary proliferation. The foamy cells were positive for periodic acid-Schiff (PAS) with diastase, oil red-O, and CD68. To our knowledge, this is the first case with foamy histiocytes without xanthomatous-appearing skin lesions in POEMS syndrome. It would be worth searching for xanthomatous histiocytes in the hyperpigmented skin of patients with POEMS syndrome.     

Granuloma annulare with a mycosis fungoides-like distribution and palisaded granulomas of CD68-positive histiocytes

We describe 3 unusual cases of granuloma annulare with multiple macular lesions in a distribution that simulated mycosis fungoides in patients with no associated underlying diseases. Repeated biopsies showed typical well-formed palisading granulomas and no evidence of an atypical lymphocytic infiltrate. There was no vasculitis, neutrophilic, eosinophilic, or interstitial infiltrate. The patients had no associated underlying diseases. Most of the histiocytes in the palisading granulomas were strongly positive for CD68. The lymphocytes were a minor component of the granulomatous inflammation and were predominantly CD8(+) T-cells. The findings in these cases add to the spectrum of previously defined granulomatous eruptions of the skin.     

Multicentric reticulohistiocytosis with elevated cytokine serum levels

Multicentric reticulohistiocytosis (MRH) is an uncommon non-Langerhans cell histiocytosis of unknown etiology. It is a multisystem disorder characterised by a papulonodular skin eruption, mainly in the extensor surfaces, and destructive polyarthritis. Histologically, either cutaneous lesions or the synovium show a dense dermal infiltrate of histiocytes and multinucleated giant cells with an eosinophilic granular material in the cytoplasm. In the immunohistochemical analysis these cells stain positively with monocyte/macrophage markers (CD68 and CD45), as well as with certain cytokines (tumor necrosis factor-α, interleukin 1β and interleukin 6). Moreover, recent reports suggest an osteoclastic nature of the infiltrating cells, as they stain strongly with osteoclast tissue lytic markers including tartrate-resistant acid phosphatase and cathepsin K. We report a case of MRH presenting with clinical features of dermatomyositis. Furthermore, the patient showed elevated cytokine serum levels that lowered after therapy.     

Progressive histiocytosis: description of a case of slow-course non-Langerhans cell histiocytosis

We describe the case of a 37-year-old male patient who in 1992 started developing a skin eruption characterized by dark red to red-yellow papulonodular lesions that showed no tendency to spontaneous resolution. Visceral organs were not involved and the lipid pattern was basically normal. Histological examination revealed an infiltrate in the mid and upper dermis mainly consisting of mononucleated and multinucleated histiocytes with an abundant eosinophilic cytoplasm and a ground glass appearance, admixed with numerous xanthomatized cells and Touton and foreign-body giant cells. Immunohistochemical studies showed positivity for CD68 and negativity for CD1a and S-100 protein, whereas at the electron microscopy level the only peculiar finding was the presence of many desmosome-like junctions. The authors believe this to be a borderline form between papular xanthoma and reticulohistiocytosis.     

Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis,granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters

The main histopathological features in the cutaneous lesions of Churg‐Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non‐vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Ishibashi M, Kudo S, Yamamoto K, Shimai N, Chen K‐R. Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters.