持久性隆起性红斑伴再生障碍性贫血一例并文献复习

持久性隆起性红斑(erythema elevatum diutinum, EED)是一种罕见的皮肤白细胞破碎性血管炎,其病因不明确,认为与感染、血液系统疾病、自身免疫性疾病感染的循环免疫复合物在血管壁沉积有关,因此除单纯的皮肤表现外,既往报道39.4%的EED伴发HIV、病毒性肝炎、链球菌感染、副蛋白血症、骨髓增生异常综合征、系统性红斑狼疮和类风湿性关节炎等疾病。现报道我院诊治的持久性隆起性红斑伴再生障碍性贫血一例,并通过文献检索,分析伴发其他系统性疾病的EED的临床特征,初步探讨EED与血液系统疾病及恶性肿瘤的关联性。     

Erythema elevatum diutinum

A 53-year-old man with human-immunodeficiency-virus infection presents with a 2-year history of nodules on the extensor surfaces of his extremities. A biopsy confirmed the diagnosis of erythema elevatum diutinum. The clinical presentation, histopathology, etiology, and treatment options are reviewed.     

Erythema elevatum diutinum

1. A typical case of Erythema elevatum diutinum (E.e.d.) with extracellular cholesterosis is described clinically and histologically in a 48-year-old woman. The disease had a course of 15 years. 2. Electron microscopy confirmed the histological and immunfluorescent findings of leucocytoclastic vasculitis. In older lesions, histiocytes/macrophages predominate. Intra- and extracellular lipid depositions showed a variety of ultrastructural characteristics which differed from electron microscopical findings described in other disorders associated with lipid deposition. 3. The findings are in accordance with the hypothesis that the primary event in E.e.d. is a special type of chronic leukocytoclastic vasculitis. Subsequently, the damage of the vessel walls and focal necrosis may lead to secondary lipid deposition.     

Erythema elevatum diutinum

Zusammenfassung Eine leukocytoklastische Vaskulitis wurde durch intrakutane Injektion von Streptokokkenantigen bei einer Patientin mit Erythema elevatum diutinum (E.e.d.) ausgelöst. Der immunelektronenmikroskopische Nachweis von C3 in dieser Reaktion wurde 24 h nach der Injektion der Antigens mit Hilfe der Peroxydase-Antiperoxydase-Mehrstufentechnik durchgeführt.C3-Niederschläge fanden sich in der Intercellularfuge zwischen Endothelzellen, Pericyten und glatten Muskelzellen und der aufliegenden Basallamina sowie zwischen den Duplikaturen der mehrschichtigen Basallamina kleiner Gefäße. Intakte und zerfallende Neutrophile durchsetzen die Gefäßwände und die Gefäßumgebung. Es resultieren Nekrose und Fibrinablagerung.Die Befunde zeigen die Sequenz der Ereignisse bei leukocytoklastischer Vaskulitis auf dem ultrastrukturellen Niveau und stützen gleichzeitig die Hypothese, daß eine durch Bakterienantigen ausgelöste Reaktion vom Arthus-Typ bei E.e.d. pathogenetisch bedeutsam ist.Stipendiat der Alexander-von-Humboldt-Stiftung aus Warschau/Polen     

Erythema elevatum diutinum

First described by Hutchinson in 1878, erythema elevatum diutinum (EED) is a rare disease characterized by persistent red, raised nodules and plaques in a symmetrical, acral distribution. The disease occurs equally in the sexes, primarily in the 30- to 60-year age group. We report a representative case of EED which responded to current agents, and briefly review the status of the disease.     

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.     

Erythema elevatum diutinum in human immunodeficiency virus-infected patients — report of a case and review of the literature

Erythema elevatum diutinum (EED) is a rare chronic disease of unknown origin, part of the spectrum of cutaneous leucocytoclastic vasculitis, A case of EED in a 32-year-old HIV-infected male patient, with no previous opportunistic infections and a CD4+ cell count of less than 200/mm³ is reported. Therapy with oral dapsone (100 mg/day for 15 days) resulted in clinical cure with no relapse after 6 months of follow-up. To our knowledge, only six cases of EED in HIV-positive patients have been reported to dale. A brief review of these seven cases is described.     

Erythema elevatum et diutinum

Erythema elevatum et diutinum (EED) is a rare, chronic, cutaneous, leukocytoclastic vasculitis. It is characterized by symmetric, plaques, papules and nodules occurring preferentially on the extensor aspects of the arms. An association with chronic infections, neoplasms, especially myeloproliferative diseases and paraproteinemia, as well as autoimmune disorders (rheumatoid arthritis, Crohn disease) has been described. An 83-year-old woman with EED presented with symmetric, skin colored, firm nodules on the extensor surfaces of her fingers for two years. This case report summarizes the central clinical aspects and differential diagnosis of EED as well as its therapeutic options.