Unique aspects of a lesion of erythema elevatum diutinum

A 70-year-old man with erythema elevatum diutinum developed new lesions on his right hand 4 years after initiation of treatment with dapsone. An unusual protruberant appearance of these lesions grossly and a peculiar fibrosing quality histopathologically of one lesion are features that, to our knowledge, have not been reported previously.     

Late-stage nodular erythema elevatum diutinum

Erythema elevatum diutinum (EED) is a rare chronic vasculitic process of unknown etiology that presents as bilateral, symmetrical, periarticular, red-brown papules and plaques, often over dorsa of joints. Early histologic changes are characterized by leukocytoclastic vasculitis. With chronicity, the lesions develop dense neutrophilic infiltrate and fibrosis. We describe an unusual case of late-stage nodular EED present for 35 years in a seronegative 75-year-old man. Asymptomatic papules and plaques measuring from 0.7 to 9.0 cm were seen over the interphalangeal joints, elbows, knees, and ankles. The histologic findings were characterized by predominant concentric fibrosis that formed well-circumscribed dermal and subcutaneous nodules. Awareness of this unusual presentation of EED helps to avoid misdiagnosis as cutaneous neoplasms.     

Vesiculobullous variant of erythema elevatum diutinum

The vesiculobullous variant of erythema elevatum diutinum (EED) is a very rare variant of EED. We describe a 16‐year‐old boy who presented with symmetrical nodular lesions accompanied by vesicles on the dorsa of his hands. Biopsy findings were consistent with EED. The histopathological presence of IgA and neutrophils in the vesicles indicates that the joint action of both is responsible for formation of these subepidermic vesicles. We hypothesize that absence of human leucocyte antigen related to dermatitis herpetiformis (DH) in our patient might have influenced the location and distribution of the lesions, so that they were not typical of DH. We report the second case of the vesiculobullous variant of EED with IgA deposits in the dermoepidermal membrane. To our knowledge, there are only 14 previously reported cases of the vesiculobullous variant of EED.     

持久性隆起性红斑一例

患者男,52岁,臀部、四肢反复红斑、丘疹、血疱伴痛1年.患者1年前无明显诱因四肢关节伸侧先后出现片状红斑、丘疹、结节,中央出现血疱、坏死、结痂,伴痛,皮疹持久不消退,部分融合扩大,且伴肘膝、掌指、踝关节疼痛,但无晨僵、关节畸形及活动障碍,无发热、肌肉酸痛.曾服用中药等治疗(具体不详),皮疹未消退.近半年臀部、手足也出现类似皮疹,关节疼痛较前加剧,指关节肿胀、压痛明显,活动受限,遂至我院住院治疗.既往体健.     

Atypical presentation of erythema elevatum diutinum

A 47-year-old man presented with two red-brown plaques on his back. The histology was consistent with erythema elevatum diutinum. The case was unusual in the site of presentation and in the paucity of lesions. Investigations were aimed at screening for associations such as infections, malignancies and inflammatory diseases. Dapsone remained an effective treatment.     

Unusual erythema elevatum diutinum with fibrohistiocytic proliferation

A 78-year-old woman with a history of symmetrical erythematous plaques on the arms, and a monoclonal gammopathy. developed a strange striped reticulate papular dermatosis with central atrophy. Histological examination was compatible with a very late stage of erythema elevatum diutinum (EED). showing a fibrohistiocytic proliferation with areas of granulation tissue. This fibrosis may result from the chronic dermal injury of leucocytoclastic vasculitis and is sometimes the predominant histology of EED. Investigations for underlying haematological anomalies, such as paraproteinaemia and infection with human Immunodeficiency virus, must be performed. Dapsone is ineffective once the fibrous nodules have appeared.     

The evolution of lesions in erythema elevatum diutinum

We obtained biopsies from early, fully developed, and late lesions of erythema elevatum diutinum (EED) in a 49-year-old man. The histologic and electron-microscopic findings were compared with those reported in the literature and three other cases from our files. Early lesions show leukocytoclastic vasculitis with capillary proliferation. Later lesions show vasculitis, dermal aggregates of neutrophils, fibrosis, and areas of granulation tissue. Newly formed vessels in granulation tissue may be more susceptible to damage by immune complexes, and the early formation of granulation tissue in EED may prevent an early resolution of vasculitis. Damage to dermal connective tissue in EED incites either scarring or, rarely, a fibrohistiocytic proliferation. Ultrastructural examination of one case showed histiocytes with myelin figures and intracellular lipid and cholesterol.     

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.     

持久性隆起性红斑伴眼部损害一例

患者男,64岁,四肢、耳廓皮损20年,右眼红、痒伴流泪5年,累及左眼2年。患者20年前四肢、耳廓皮肤出现红斑、结节,伴疼痛,皮损逐渐加重,就诊于吉林大学第一医院皮肤科等多家医院,曾用左氧氟沙星及地塞米松静脉滴注（剂量不详）,余治疗药物不详,病情好转,停药后复发。其后患者用多种中药等治疗,皮损无好转。患者曾就诊于北京协和医院,行皮肤病理活检,诊断为持久性隆起性红斑,建议应用氨苯砜治疗。因门诊缺药,给予雷公藤及其他药物口服（不详）,皮损减轻,但仍反复发作……     

甲氨蝶呤治疗持久性隆起性红斑1例

患者女,37岁,因四肢斑块伴胀痛半年,加重2周于2020年1月来我所就诊。半年前患者无明显诱因四肢出现红斑,渐增多变大,伴疼痛。于外院行病理检查,示右小腿皮损真皮乳头水肿不明显,真皮全层及皮下脂肪血管丛周围见大量中性粒细胞、淋巴细胞浸润,可见核尘,血管纤维样改变不明显……     

A case of erythema elevatum diutinum associated with breast carcinoma

A 53-year-old woman diagnosed with invasive ductal-type breast carcinoma was referred to our clinic with red–purple lesions on the hands and legs. She had neither pruritus nor pain. The first lesion developed on the dorsal hand. In the following days, new lesions appeared on the extensor surface of the legs. The patient had been treated with modified radical mastectomy and three courses of cyclophosphamide, adriamycin, and fluorouracil chemotherapy. Dermatologic examination revealed reddish-violaceous papules and plaques ranging from a few millimeters to 2 cm in diameter, bilaterally located on the dorsal hands, especially over the metacarpophalangeal and interphalangeal joints ( Fig. 1 ). Multiple red–purple, circumscribed papules and plaques of various diameters were observed bilaterally over the shins ( Fig. 2 ). The largest of these plaques showed an annular configuration. The nails showed distal subungual keratosis and yellow discoloration. The rest of the physical examination was normal.

Figure 1 Open in figure viewer PowerPoint Violaceous papules and plaques on the dorsal hands     

氨苯矾治愈持久隆起性红斑一例报告

患者男,11岁.3年前秋于面部两手背掌指关节处对称性发生红斑,约蚕豆大,高起皮面,微痒,后发展至两肘、髋、膝及两躁关节处,冬重夏轻,皮疹持续时间长,可消退,但新皮疹相继发生,从不间断.病后服强的松等均无效.     

Infantile erythema elevatum diutinum: report of a vesiculo-bullous case

Erythema elevatum diutinum (EED) is a localized, low-grade form of leukocytoclastic vasculitis mainly affecting adults, with only a few pediatric cases reported in the literature. The pathogenesis of this dermatosis is unknown, although an Arthus-type reaction to bacterial and viral antigens seems involved. We describe a case of EED in a 9 year-old child suffering from recurrent pharyngotonsillitis induced by Streptococcus pyogenes. Clinical presentation of the dermatosis was atypical, with vesicles and bullae intermingled with red-purple papules and plaques distributed mainly over the extensor aspects of the extremities. Histopathological examination of a vesiculo-bullous lesion revealed a subepidermal bulla containing neutrophils and nuclear dust suggesting dermatitis herpetiformis. Fibrin in vessel walls was observed throughout the dermis. Direct immunofluorescence was negative. A prompt and complete response to sulphone therapy was obtained in conjunction with tonsillectomy. Clinicians should be aware of atypical presentations of EED in children with vesicles and bullae mimicking During's disease.     

Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal gammopathy

A patient with erythema elevatum diutinum (EED) developed pyoderma gangrenosum (PG). Investigation revealed an IgA kappa monoclonal gammopathy. Previous reports of PG in association with EED are reviewed and the spectrum of the neutrophilic dermatoses discussed.