IgG4相关性皮肤病

报告IgG4相关性皮肤病(IgG4-RSD)1例。患者男,25岁。躯干、面颈部瘙痒性红色至褐色丘疹、斑块及结节6年。皮损组织病理示真皮内胶原纤维增生,细血管周围小团块状或结节状淋巴细胞、浆细胞浸润。免疫组化示浆细胞表达CD38、CD138、CD19、κ-轻链、λ-轻链、IgG、IgG4均阳性,IgG4+/IgG+浆细胞比值40%,每高倍视野下IgG4+浆细胞10个。该文对IgG4-RSD的诊断、鉴别诊断和治疗进行讨论。     

IgG4相关性疾病的研究进展

IgG4相关性疾病是近年来提出的一种新病种,本病可累及人体任何组织和器官.IgG4相关性疾病的显著特征为血清IgG4水平升高以及多种器官和组织中IgG4阳性浆细胞浸润.近年来发现,IgG4相关性疾病可有皮肤改变,皮损可表现为多种皮肤病的症状,包括Mikulicz病、皮肤浆细胞增多症、假性淋巴瘤、血管淋巴样增生伴嗜酸细胞增多和木村病等.IgG4相关性疾病患者对系统糖皮质激素治疗反应较好.     

皮肤浆细胞增多症七例临床病理分析北大核心CSCD

目的 探讨皮肤浆细胞增多症临床和组织病理表现。方法 对7例皮肤浆细胞增多症进行临床病理分析。结果 7例患者中女2例,男5例,平均年龄42.4岁,病程2 ~ 10年。临床表现为缓慢进展、多发的棕色斑片及斑块。7例患者血尿粪常规、心电图和腹部B超等检查未见异常。3例行血清IgG检测,均高于参考值。2例做骨髓细胞学检查,浆细胞比例增高占5％,以成熟浆细胞为主。7例皮损组织病理：表皮基底层色素增加,真皮血管周围见以淋巴细胞、成熟浆细胞为主的浸润。免疫组化结果示,7例κ和λ轻链表达比例均近似1∶1,IgG均为强阳性表达,1例个别浆细胞IgG4阳性,6例IgG4阴性。结论 皮肤浆细胞增多症病因不明,有独特的临床及病理表现。     

IgG4相关的硬化性疾病

IgG4相关硬化性疾病是近年被认识的一种疾病,被认为与自身细胞、体液免疫反应异常有关。患者血清IgG4水平升高,病变器官纤维化,并有显著IgG4阳性浆细胞浸润。病变可以累及许多器官,部分病例伴有皮肤的病变。皮肤病变以头颈部多见,可为红斑、斑片或皮下结节,有痒感。IgG4相关皮肤疾病糖皮质激素治疗有效。     

IgG4相关性疾病的皮肤表现

IgG4相关性疾病是近期被认识的一种系统疾病。其临床表现为多器官进行性受累,外周循环中可检出高滴度的IgG4。该病可以有皮肤受累,根据皮损中是否有大量浆细胞浸润,皮疹可以分为原发性和继发性两大类,原发性皮疹包括皮肤浆细胞增多症,假性淋巴瘤、伴嗜酸性粒细胞增多的血管淋巴样增生和米库利奇病。继发性皮疹包括银屑病样斑块、非特异性斑丘疹和红斑、瘙痒性皮疹、紫癜性皮疹和末端指关节缺血。该文描述了Ig G4相关性疾病的皮肤表现,并对其发病机制和鉴别诊断进行了探讨,以加深对该类疾病的认识,达到早期诊断和干预的目的。     

皮肤浆细胞增多症

报告1例皮肤浆细胞增多症.患者男,42岁.皮损表现为淡红褐色斑块及结节,累及头面部、躯干、腋窝及腹股沟.血IgG及IgA增高.皮损组织病理检查示真皮血管周围以成熟浆细胞为主的浸润.免疫组化检查k和入轻链均阳性,证实浸润的浆细胞为多克隆增殖.诊断:皮肤浆细胞增多症.该病需与其他浆细胞增多性疾病相鉴别.     

关节病性银屑病合并IgG4相关性腮腺炎1例

患者女,50岁,双手反复疼痛3年,加重伴双侧下颌包块1年。患者头皮以及腰骶部散在红色斑块,其上可见银白色鳞屑、薄膜以及点状出血现象;双手远端指间关节畸形;颌下以及耳后可触及肿大淋巴结;右侧腮腺淋巴结病理活检示:纤维组织增生伴较多浆细胞及淋巴细胞浸润;免疫组织化学示:IgG4/IgG40%,IgG440个/HPF。诊断:IgG4相关性腮腺炎。     

皮肤浆细胞增多症

报告1例皮肤浆细胞增多症.患者男,51岁.右下肢股外侧浸润性红斑4年余,口周和腹部皮损1年.皮损组织病理检查:真皮深层及部分皮下组织可见以成熟浆细胞为主的大量炎性细胞浸润,其间有少量淋巴细胞及多核巨细胞.免疫组化染色示:浸润的浆细胞CD79a、CD138阳性.诊断:皮肤浆细胞增多症.     

浆细胞性唇炎1例

报告1例浆细胞性唇炎。患者女,78岁,右上唇持续性红色斑块2年,斑块质地柔软,界限清楚,表面轻度糜烂。皮肤组织病理显示粘膜固有层多量浆细胞浸润。诊断为浆细胞性唇炎。     

浆细胞性龟头炎一例并文献复习

本文报道1例浆细胞性龟头炎患者并回顾相关文献。患者,男,65岁。龟头红斑2年,皮疹表现为龟头两处境界清楚呈花瓣型鲜红色斑片,表面湿润,触诊无压痛,无明显溃疡,无明显浸润感。皮肤组织病理检示：表皮萎缩,部分真表皮分离,真皮浅层大量淋巴细胞、浆细胞浸润。诊断:浆细胞性龟头炎。治疗：外用1%吡美莫司乳膏,3个月后皮疹好转,现正随访中。     

IgG4-positive plasma cells in cutaneous Rosai-Dorfman disease: an additional immunohistochemical feature and possible relationship to IgG4-related sclerosing disease

Background: Cutaneous Rosai-Dorfman disease (CRDD) shares the histopathological features of abundant plasma cells and stromal fibrosis with IgG4-related sclerosing disease. The possible role of IgG4+ plasma cells in CRDD was investigated.
Methods: Twelve cases of CRDD were reviewed, and their lesions were immunostained with anti-IgG4 and anti-IgG antibodies. The number of IgG4+ and IgG+ plasma cells and their ratios were estimated. Serum IgG4 and IgG concentrations were measured in two recent cases.
Results: Many IgG4+ and IgG+ plasma cells were found in all 12 cases. IgG4+ plasma cells ranged from 21 to 204 per high-power field (HPF) (mean 117/HPF), and IgG+ plasma cells ranged from 114 to 759/HPF (mean 349/HPF). All cases had more than 30 IgG4+ cells/HPF, except one case. The IgG4/IgG ratio ranged from 16% to 51% (mean 34%). Serum IgG4 concentration and serum IgG4/IgG ratio were increased in one recent case. Various degrees of stromal fibrosis were present in all cases.
Conclusions: The presence of many IgG4+ plasma cells and stromal fibrosis suggests that CRDD may be related to IgG4-related sclerosing disease. Many IgG4+ plasma cells is another feature of CRDD, and serum IgG4 may be elevated.     

IgG4-related skin disease, a mimic of angiolymphoid hyperplasia with eosinophilia

Mikulicz's disease is considered one of the IgG4-related diseases that are characterized by elevated serum IgG4 concentrations and the immunohistochemical finding of IgG4-positive plasma cells. The IgG4-related diseases often exhibit a wide variety of eosinophil infiltration. A 66-year-old male with Mikulicz's disease developed multiple, nonpruritic, red papules on the left opisthotic region 2 years after diagnosis. A biopsy of the skin lesions revealed follicle-like formation in the dermis and subcutaneous tissue containing nodular lymphocytic infiltration with numerous eosinophils and plasma cells, predominately around venules, mimicking angiolymphoid hyperplasia with eosinophilia (ALHE). Immunohistochemically, most IgG-expressing plasma cells were positive for IgG4 (IgG4/IgG ratio = 72%). Our patient appeared to have a condition associated with the IgG4-related diseases. Caution should be exercised in diagnosing skin lesions of the IgG4-related diseases, which are confusingly similar in appearance and histology to ALHE.     

IgG4‐related skin disease

IgG4‐related disease (IgG4‐RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4⁺ plasma cells. IgG4‐RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4‐RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4‐mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis‐like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1–3 are induced by direct infiltration of IgG4⁺ plasma cells, while the other types (4–7) are caused by secondary mechanisms. IgG4‐related skin disease is defined as IgG4⁺ plasma‐cell‐infiltrating skin lesions that form plaques, nodules or tumours (types 1–3), but may manifest secondary lesions caused by IgG4⁺ plasma cells and/or IgG4 (types 4–7).     

Skin lesions in a patient with IgG4‐related disease

IgG4‐related disease (IgG4‐RD) is a newly recognized condition that is characterized by raised levels of serum IgG4, tissue infiltration of IgG4‐positive plasma cells and presence of fibrosis. It affects multiple organs, including the pancreas, bile duct, and lacrimal and salivary glands. Skin lesions have rarely been reported, and those that have were described as distributed mainly in the head and neck region. We report a case of IgG4‐RD with autoimmune pancreatitis and skin lesions on the trunk and limbs. The skin lesions responded well to oral prednisolone (PSL); however, tapering of PSL to 5 mg/day resulted in recurrence. At present, the skin disease is well controlled at a dose of 7 mg/day. Interestingly, IgG4 levels fluctuated with changes in the PSL dose but did not coincide with the severity of the skin disease, implying that the raised levels of IgG4 did not directly influence the skin symptoms.     

Case of immunoglobulin G4‐related skin disease: Possible immunoglobulin G4‐related skin disease cases in cutaneous pseudolymphoma only by immunohistochemical analysis

Immunoglobulin G4‐related disease (IgG4‐RD) is a newly recognized disease characterized by elevated serum IgG4 levels, tissue infiltration rich in IgG4⁺ plasma cells. We report on a case which was first considered as pseudolymphoma from the histopathological analysis, but finally diagnosed as IgG4‐related skin lesions. As the morphological features of cutaneous involvement of IgG4‐RD are consistent with those of cutaneous pseudolymphoma, we tried immunostaining past potential cases of IgG4‐RD. Thirty‐two skin specimens (15 men and 17 women; mean age, 53 years) diagnosed as having pseudolymphoma were retrieved from the archives to conduct hematoxylin–eosin, IgG and IgG4 staining. Out of the 32 cases of cutaneous pseudolymphoma, germinal center formation was seen in 22 cases, and moderate–severe fibrosis was seen in seven cases. Eleven cases showed more than 10 IgG4⁺ plasma cell infiltration/high‐power field, and among these 11 cases, seven cases (22%) showed A ratio of IgG4⁺/IgG⁺ cells of more than 40%. Thus, out of the 32 cases of cutaneous pseudolymphomas, two cases (6.3%) satisfied IgG4‐RD histopathological diagnostic criteria. As clinical presentations and histopathological features of skin involvement of IgG4‐RD are analogous to cutaneous B‐cell pseudolymphoma, careful identification is required through systemic examination, serum IgG4 measurement and other means.     

Immunoglobulin-G4–related skin disease

Immunoglobulin-G4–related disease (IgG4-RD) is an autoimmune-mediated spectrum of diseases, characterized by infiltration of IgG4+ plasma cells into one or multiple organs, with the pancreas being the most commonly affected organ. The disease mostly affects middle-aged to elderly men. Diagnosis requires an integration of clinical, radiologic, pathologic, and serologic studies. Histologically, there is an increased infiltration of IgG4+ plasma cells, elevated ratio of IgG4+/IgG plasma cells of more than 40%, and a storiform pattern of fibrosis. There may be eosinophilia, along with elevated IgG4 levels. IgG4-RD can mimic several diseases and should be differentiated from inflammatory and neoplastic processes. Recently, there has been increased awareness of cutaneous involvement by IgG4-RD either as an isolated lesion or primary involvement or as a secondary involvement from a systemic disease. Clinically, cutaneous IgG4+-related disease presents as papules, plaques, and nodules involving the head and neck areas. We have provided a systematic review of the literature of this new and challenging entity of cutaneous IgG4-RD.     

Immunoglobulin G4‐related disease presenting with prurigo: Circulating T‐helper 2 cells may be involved in the pathogenesis

We report a case of immunoglobulin G4‐related disease (IgG4‐RD) which presented with prurigo on the trunk and extremities. A 66‐year‐old man had a 2‐month history of itchy erythematous papules on his trunk and extremities. Bilateral eyelid swelling and enlargement of the submandibular and parotid glands were also observed. Computed tomography revealed pleural thickening and diffuse pancreatic enlargement. Serum levels of IgG4 were markedly increased. A biopsy specimen obtained from an erythematous papule showed a perivascular inflammatory infiltrate of lymphocytes with eosinophils in the dermis, whereas a parotid gland biopsy revealed an infiltrate of abundant IgG4‐positive plasma cells. Treatment with prednisolone resulted in improvement of the skin and other lesions along with a decrease in IgG4 serum levels. A flow cytometric assay revealed that percentages of interleukin (IL)‐4‐ and IL‐13‐producing CD4⁺ T cells were markedly higher in the circulation of the IgG4‐RD patient than in that of healthy subjects. Moreover, those populations dramatically decreased after treatment. Thus, prurigo may be a skin manifestation of IgG4‐RD and T‐helper 2 cells may contribute to the pathogenesis.