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[环状弹性组织溶解性巨细胞肉芽肿]

[Annular elastolytic giant cell granuloma].

作者信息

Djilali-Bouzina F, Grange F, Krzisch S, Schnebelen M-P, Grosshans E, Guillaume J-C

机构信息

Service de dermatologie, hôpital Pasteur, Colmar cedex, France.

出版信息

Ann Dermatol Venereol. 2010 Aug-Sep;137(8-9):536-40. doi: 10.1016/j.annder.2010.06.004. Epub 2010 Aug 19.

Abstract

BACKGROUND

Annular elastolytic giant cell granuloma (AEGCG) is a rare form of granulomatous dermatosis. It is characterised histologically by phagocytosis of elastic fibres by multinucleated cells. We report a favourable outcome in a case of AEGCG following PUVA therapy and treatment with synthetic antimalarials.

PATIENTS AND METHODS

A 67-year-old retired wine grower presented with highly pruritic annular lesions with raised borders on the shoulders and trunk that had been present for several months. Histological examination of a biopsy sample from the erythematous border was characteristic of AEGCG. Various topical treatments proved ineffective and systemic corticosteroids attenuated the patient's pruritus but had no effect on the skin lesions. PUVA therapy resulted in regression of lesions on the trunk, but the rash spread to the patient's arms and was covered with epidermal microcysts. PUVA therapy was discontinued and treatment with a synthetic antimalarial (hydroxychloroquine 400mg/d) was initiated, resulting in complete regression of the lesions.

DISCUSSION

AEGC was isolated in 1979 by Hanke et al. on the basis of five cases seen in females. This is a rare form of dermatosis with some 30 cases being reported in the English literature. The clinical aspect is fairly evocative, with erythematous papular lesions, either alone or in groups, with a raised border and a lighter centre tending towards atrophy. In most cases, the lesions are found predominantly in areas exposed to sunlight. The histological appearance is characteristic, with an image of giant cell elastophagic granuloma from which the name of the disease is taken. This appearance allows the disease to be differentiated from a number of other granulomatous diseases. The aetiology is unknown and treatment is empirical. Spontaneous cure can occur and consistent results have not been obtained with any treatments. In our case, PUVA was partly successful, and the synthetic antimalarials resulted in complete regression of residual lesions.

摘要

背景

环状弹性组织溶解性巨细胞肉芽肿(AEGCG)是一种罕见的肉芽肿性皮肤病。其组织学特征为多核细胞吞噬弹性纤维。我们报告了1例AEGCG患者在接受补骨脂素加紫外线A(PUVA)疗法及合成抗疟药治疗后取得良好疗效的病例。

患者与方法

一名67岁的退休葡萄种植者,肩部和躯干出现边界隆起的高度瘙痒性环状皮损,已持续数月。对红斑边界处活检样本进行组织学检查,结果符合AEGCG特征。多种局部治疗均无效,全身使用皮质类固醇可减轻患者瘙痒,但对皮肤损害无作用。PUVA疗法使躯干皮损消退,但皮疹蔓延至患者手臂,且出现表皮微囊肿。停用PUVA疗法,开始使用合成抗疟药(羟氯喹400mg/d)治疗,皮损完全消退。

讨论

1979年汉克等人根据5例女性病例首次发现AEGC。这是一种罕见的皮肤病,英文文献中报道约30例。其临床表现颇具特征性,表现为红斑丘疹性皮损,可为单个或成群,边界隆起,中央颜色较淡且有萎缩倾向。多数情况下,皮损主要见于暴露于阳光的部位。组织学表现具有特征性,呈现巨细胞弹性组织吞噬性肉芽肿图像,该病即由此得名。此表现有助于将该病与其他多种肉芽肿性疾病相鉴别。病因不明,治疗为经验性。疾病可自愈,且任何治疗方法均未取得一致的疗效。在我们的病例中,PUVA疗法部分成功,合成抗疟药使残留皮损完全消退。

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