持久性隆起性红斑8例临床及组织病理特点分析

目的 探讨持久性隆起性红斑(erythema elevatum diutinum, EED)的临床特征及病理特点。方法 回顾性分析2011年2月-2021年4月在本院皮肤科收治的8例EED患者的临床及病理资料。结果 临床上8例患者均表现为四肢伸侧丘疹、结节或斑块，其中2例合并有红斑、水疱或脓疱。伴发疾病包括原发性胆汁性胆管炎、浆细胞性骨髓炎、类风湿关节炎、间质性肺炎、肺结核、前列腺结核、隐性梅毒、前列腺癌、急性肾损伤、高血压、糖尿病等。皮损组织病理：红斑、水疱处表现为白细胞碎裂性血管炎，斑块、结节性皮损处主要为真皮全层血管壁纤维素样变性，真皮内毛细血管及胶原纤维增生。结论 氨苯砜治疗早期EED病变最有效，同时需注意EED合并系统疾病的诊治及随访。     

伴发溃疡的持久性隆起性红斑北大核心CSCD

报告1例伴发溃疡的持久性隆起性红斑。患者男,59岁。全身反复结节、斑块伴溃疡15年。皮损组织病理检查示浅表糜烂结痂,部分表皮缺失,真皮上部明显水肿,血管周围及胶原束间可见大量中性粒细胞及淋巴细胞为主的炎性细胞浸润,血管内皮细胞肿胀,明显核碎裂。诊断:持久性隆起性红斑。     

持久性隆起性红斑:皮肤和系统性表现、免疫学研究及氨苯砜的成功治疗

持久性隆起性红斑(Erythema elevatum diutinum,EED)是一种罕见的慢性皮肤病,其特点为持久性红色、紫红色和淡黄色丘疹、斑块和结节,通常对称地分布于肢端的伸面。组织学改变为白细胞碎裂性脉管炎,在真皮上部和中部的血管壁伴有纤维蛋白样坏死。以往的文献复习和病例报告强调以下几点:1.无系统性侵犯;2.偶尔发生关节疼痛;3.尚无成功的疗法,虽然已报告有几例病     

持久性隆起性红斑1例

报告持久性隆起性红斑1例。患者男，51岁，汕头人。面部、躯干、四肢关节伸侧出现紫红色结节、斑块伴轻度瘙痒1年半。皮肤组织病理示：表皮正常，真皮中、上部弥漫性嗜中性粒细胞浸润伴嗜酸性粒细胞和少许淋巴细胞，个别血管壁纤维蛋白样变性，血管壁及血管周可见嗜中性白细胞浸润、核尘及管外红细胞。诊断符合持久性隆起性红斑。     

持久性隆起性红斑并发溃疡1例

持久性隆起性红斑是一种白细胞碎裂性血管炎,临床上不常见,诊断主要依靠临床表现、组织病理及免疫组织病理的结果.笔者诊治1 例伴有溃疡的持久性隆起性红斑患者,经治疗后溃疡已愈合,皮损基本消退,现报告如下.     

泛发性持久性隆起性红斑

报告1例全身泛发的持久性隆起性红斑.患者男,36岁.全身反复红色丘疹、水疱、结节伴瘙痒1年余.皮损组织病理示白细胞碎裂性血管炎.诊断:持久性隆起性红斑.     

氨苯砜单一疗法治疗持久性隆起性红斑一例并文献复习

报道1例氨苯砜单一疗法治疗持久性隆起性红斑并对既往报道文献进行复习。患者，女，80岁。双手掌、臀部、双下肢及足底红褐色斑片半年。组织病理检查：表皮角化过度、角化不全，棘层轻度增厚，真皮浅中层血管壁纤维蛋白样变性，血管周围轻度嗜中性粒细胞，少许淋巴细胞浸润，局部胶原纤维嗜碱性变。诊断：持久性隆起性红斑。口服氨苯砜100 mg,日1次，3个月后随访，皮损明显好转。     

结节性持久性隆起性红斑1例

持久性隆起性红斑(erythema elevatum diutinum,简称EED)是一种慢性纤维化性白细胞碎裂性血管炎,其典型皮损为四肢关节伸侧的紫红色斑块,而表现为大结节者则很少见[1],现将我科诊治的1例报告如下. 1 病历摘要 患者女,70岁.因全身起多发性皮肤结节伴疼痛3年,于2010年5月27日来我科门诊就诊.3年前,无明显诱因患者双足后跟出现结节,伴轻微疼痛,在当地医院行手术切除,组织病理检查未明确诊断.此后,右足趾端伸侧、左手掌指关节、指间关节伸侧、双肘关节伸侧、右耳和臀部陆续出现类似结节伴疼痛,受压部位的皮损表面有破溃.在当地医院行数次病理检查均未明确诊断.患者既往体健,否认有关节痛、结核病等病史,无皮肤及内脏肿瘤病史.家族中无类似疾病患者.     

持久性隆起性红斑伴特发性血小板减少症及糖尿病1例

持久性隆起性红斑是临床上少见且易误诊的一种慢性局限性白细胞碎裂性血管炎,本病可并发其他系统性疾病,笔者曾诊治1例伴特发性血小板减少症及糖尿病的患者,现报道如下.     

持久性隆起性红斑伴复发性多关节炎

报告1例伴多关节炎的持久性隆起性红斑.患者女,19岁.因臀部和四肢伸侧反复出现丘疹、结节和脓疱伴多关节疼痛5年就诊.皮损组织病理示血管壁纤维蛋白样坏死,真皮血管周围有核尘和大量中性粒细胞浸润等典型的白绌胞碎裂性血管炎的改变.还可见少量中性粒细胞在真皮乳头形成微脓肿.直接免疫荧光检查在皮肤各层未见IgG、IgA、IgM和C3沉积.诊断:伴复发性多关节炎的持久性隆起性红斑.     

Erythema elevatum et diutinum as a systemic disease

Erythema elevatum et diutinum (EED) is a rare, chronic dermatosis. It has been associated with extracutaneous findings, including arthralgias, scleritis, panuveitis, peripheral ulcerative keratitis, oral and penile ulcers, and neuropathy. Additionally, EED is connected with various systemic diseases, including HIV, IgA paraproteinemia, myelomas, neutrophilic dermatoses, and inflammatory bowel diseases. The presence of such extracutaneous manifestations in EED patients suggests that EED may be a multiorgan entity. Extracutaneous manifestations in EED may involve deposition of circulating immune complexes; thus, patients with EED should be evaluated for systemic manifestations to ensure targeted management.     

Erythema elevatum diutinum: clinical, histopathologic, and immunohistochemical characteristics of six patients

Erythema elevatum diutinum (EED) is a chronic cutaneous vasculitis occurring in association with a variety of conditions including autoimmunity, infectious disease, and hematological abnormalities. The role of associated medical problems is controversial, and the exact pathogenesis of EED is unknown. A series of six cases is reported. The typical clinical presentation was that of erythematous papules and plaques involving the extensor surfaces of the extremities. Histologically, a spectrum from leukocytoclastic vasculitis to vessel occlusion and dermal fibrosis was seen. The lesions of EED have many mimics clinically and histologically. Establishing the diagnosis of EED is important so appropriate screening for associated conditions can ensue. The vascular endothelium of EED stains positive for CD31, CD34, VEGF, and factor VIIIa and negative for factor XIIIa, TGFB, and LANA, a reaction pattern that does not distinguish it from similar appearing lesions. Thus, the chronic and recurrent nature of EED is the primary means of distinguishing it from entities that are clinically and histologically similar.     

Erythema elevatum diutinum: a review of presentation and treatment

Erythema elevatum diutinum (EED) is a rare, chronic and treatable skin condition. It has many histological mimics and is often associated with a variety of underlying systemic diseases, when these are present the management and prognosis dictates the course of the EED. This review aims to highlight the differential diagnosis, clinical manifestations of EED and the possible underlying systemic disease. It is important for clinicians to be aware that EED may predate underlying conditions and the presence of lesions may indicate underlying disease activity. In some cases one may need ‘search’ for underlying disease. Treatment of these lesions is notoriously difficult. Dapsone is used as the mainstay of treatment, however other options exist. We have highlighted different treatment options and suggested a treatment algorithm. In some cases, treatment may need to be targeted at underlying disease.     

A case of erythema elevatum diutinum associated with B-cell lymphoma: a rare distribution involving palms, soles and nails

We report a case of erythema elevatum diutinum (EED) in association with malignant B-cell lymphoma. A 62-year-old man developed EED with an unusual distribution involving the palms, soles and nails. Treatment with dapsone was effective for his skin and nails until he developed generalized lymphadenopathy which turned out to be malignant lymphoma. Many haematological diseases, e.g. IgA paraproteinaemia and myeloma, have been reported in association with EED, but not malignant lymphoma. Even though it may just be a coincidence, we would like to add malignant lymphoma as one of the diseases associated with EED because the activity of EED and malignant lymphoma fluctuated in parallel.     

Kutane Vaskulitiden

Vasculitis is characterized by an inflammatory reaction of vessel walls with damage to the dependent tissues. Forms of vasculitis which frequently have skin changes include leukocytoclastic angiitis (LcV), Henoch-Schönlein purpura (HSP), cutaneous polyarteriitis nodosum (cPAN), erythema elevatum et diutinum (EED) and urticarial vasculitis (UV). In other forms of vasculitis, systemic manifestations predominate but there are a variety of skin changes. Kawasaki disease (MK), cryoglobulinemic vasculitis (kV), Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangitis (MPA) belong to this group. The causes of vasculitis are heterogeneous. Triggers include infections, drugs, collagen vascular diseases, autoimmune diseases and lymphoproliferative disorders. Idiopathic vasculitis, particularly LcV and EED, occur only once and have a self-limited course. The diagnostic work up depends on the clinical picture and includes inflammatory markers, circulating immune complexes, different types of cryoglobulins and anti-neutrophilic cytoplasmic antibodies, collagen vascular disease specific autoantibodies and additional hematological studies. Vasculitis can manifest in many organs and requires a thorough work up specifically in cases where WG, MPA, CSS and PAN are under consideration.     

Erythema elevatum diutinum: a case report and review of literature

Erythema elevatum diutinum (EED) is a rare cutaneous leukocytoclastic vasculitis thought to be related to increased levels of circulating antibodies. It has been shown to be associated with HIV infection, tuberculosis, as well as various autoimmune diseases. A retrospective review of all cases of EED indexed in PubMed between 1990 and 2014 was performed. Inclusion criteria for articles was availability of full text in English and a biopsy-confirmed diagnosis of EED. All other articles were excluded. Cases were stratified by age and anatomic location of the lesions. Treatment response was coded as “complete,” “partial,” and “none.” A total of 133 cases of EED with 381 lesions detailed in case reports and case series were included. Twenty-one cases were associated with HIV. Of 47 patients with reported paraproteinemias, IgA paraproteinemia was found in 57.45%, IgG paraproteinemia in 29.8%, IgM paraproteinemia in 10.6%, and IgD paraproteinemia in 2.1% of cases. Of 40 (30.1%) patients with reported comorbid autoimmune disease, rheumatoid arthritis was associated with 10 cases. Cancer was found to be associated with 9.77% of cases. Seventy-five patients were treated with dapsone, with 36 (48%) achieving complete treatment response, 24 (32%) achieving partial response, and seven (9.3%) achieving no response. Keeping the clinical associations of EED in mind, especially malignancy, is critical in management of the disease. More structured studies need to take place in order to fully define the mechanisms and strength of these associations.     

Erythema elevatum diutinum: an "idiopathic" case

Erythema elevatum diutinum (EED) is a rare condition with an unclear pathogenesis. Initially classified within neutrophilic dermatoses, it is now considered as a leukocytoclastic vasculitis accordingly to its histopathologic pattern. Several clinical presentations as well as many associated diseases are reported in the literature. We report a new case of EED in a 58-year-old man who presented with a three-month history of plaques and nodules on the extensor surfaces of hands, elbows, knees, ankles, forearms, and buttocks. Histology showed a leucocytoclastic vasculitis, suggestive of the diagnosis of EED. Screening for an associated pathology, namely a paraproteinemia or a solid cancer, was negative. Treatment with dapsone leads to amelioration within few weeks.     

Erythema elevatum diutinum and HIV infection: a report of five cases

Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis, 11 cases having so far been reported in the medical literature and five patients with the disease having been seen by us during the last 4 years. As the disease is poorly known, it is easily confused with Kaposi's sarcoma or bacillary angiomatosis, but the histopathological features are diagnostic. EED is considered to be an immune complex-mediated vasculitis. A streptococcal infection seemed to be the trigger factor in four of our patients. Partial control of the cutaneous lesions was achieved by the use of antibiotics.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

Erythema elevatum diutinum – a chronic leukocytoclastic vasculitis microscopically indistinguishable from granuloma faciale?

Background: As the sequential inflammatory changes are the same in erythema elevatum diutinum (EED) and granuloma faciale (GF), histopathologic distinction may be difficult. Methods: All available cases from 1998 to 2009 with the diagnosis of EED and GF were collected and reviewed, both clinically and histopathologically. Nine cases of EED and 41 cases of GF were reviewed in a blinded fashion using a checklist of 26 histopathologic criteria. Results: Only four of the evaluated criteria showed differences between GF and EED. High density of the infiltrate was noted in 97% of cases of GF but only in 56% of cases of EED. Eosinophils were the predominant cell type in 59% of cases of GF but in none of the cases of EED. Plasma cells were more frequent in GF (64%) than in EED (22%), and granulomas were never found in GF but in 22% of EED. A zone of perijunctional sparing (Grenz zone) was observed in about three quarters of the cases in both the groups. Conclusions: The histopathology of GF and EED is very similar and overlapping. The presence of a Grenz zone and patterned fibrosis does not distinguish the two diseases. However, granulomatous nodules are only seen in EED, and a predominance of eosinophils in the infiltrate favors a diagnosis of GF. Ziemer M, Koehler MJ, Weyers W. Erythema elevatum diutinum – a chronic leukocytoclastic vasculitis microscopically indistinguishable from granuloma faciale?