Amyloidosis cutis dyschromica.
Amyloidosis cutis dyschromica.
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DOI：
10.1186/1750-1172-7-95
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发表时间：
2012-12-12
影响因子：
3.7
通讯作者：
Yao H
中科院分区：
文献类型：
--
作者：
Qiao J;Fang H;Yao H
色素异常性皮肤淀粉样变性是一种罕见的皮肤淀粉样变性的变种。迄今为止，仅报告了26起案件。本研究的目的是改善这种淀粉样变性的临床和组织病理学资料，并突出这种疾病的免疫组化特征。还审查了已发表的病例。我们在一个中心对色素异常性皮肤淀粉样变性患者进行了回顾性分析。记录并分析其临床、组织病理学及免疫组化特征。本文报告10例色素异常性皮肤淀粉样变性。其中6人是女性。5例患者来自同一家族，另5例为散发。临床表现的显著特征包括全身性斑点状色素过度和色素减退斑，无症状或轻度过敏。典型的病变发生在儿童期（n = 7），偶尔在青春期后（n = 3）。在这些色素异常性皮肤淀粉样变性病例中未观察到系统性淀粉样变性沉积的证据。在乳头状真皮中观察到淀粉样沉积物，刚果红染色呈阳性。免疫组织化学研究显示淀粉样蛋白表达细胞角蛋白CK34 β E12和CK5/6。我们描述了迄今为止最大规模的色素异常性皮肤淀粉样变性系列，并回顾了已发表的患者。这种罕见的疾病的特点是广泛的斑点状高色素和低色素病变，它是一种罕见的原发性皮肤淀粉样变性的变种，没有证据表明系统性淀粉样蛋白沉积。色素异常性皮肤淀粉样变性中细胞角蛋白CK34 β E12和CK5/6的阳性染色表明淀粉样蛋白来源于角质形成细胞。
Amyloidosis cutis dyschromica is a rarely documented variant of cutaneous amyloidosis. To date, only 26 cases have been reported. The purpose of this study was to improve the clinical and histopathological data for this variant of amyloidosis and to highlight the immunohistochemical features of the disease. The published cases were also reviewed. We performed a retrospective review of patients with amyloidosis cutis dyschromica in a single centre. The clinical, histopathological and immunohistochemical features were documented and analysed. We described 10 cases of amyloidosis cutis dyschromica. Six of them were female. Five patients were from the same family, and the other 5 were sporadic. The distinguishing features of the clinical presentation included generalised mottled hyper- and hypopigmented macules, which were asymptomatic or mild pruritic. The typical onset of the lesions occurred in childhood (n = 7) and occasionally after puberty (n = 3). No evidence of systemic amyloidosis deposition was observed in these cases of amyloidosis cutis dyschromica. Amyloid deposits were observed in the papillary dermis and were positive for the Congo red stain. An immunohistochemical study showed that the amyloid expresses cytokeratins CK34βE12 and CK5/6. We described the largest series of amyloidosis cutis dyschromica to date and reviewed the published patients. This rare disease is featured by generalised mottled hyper- and hypopigmented lesions, and it is a rare variant of primary cutaneous amyloidosis without evidence of systemic amyloid deposition. Positive staining for the cytokeratins CK34βE12 and CK5/6 in amyloidosis cutis dyschromica suggests that the amyloid is derived from keratinocytes.
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影响因子：
3.1
作者：
Chandran, Nisha Suyien;Goh, Boon-Kee;Goh, Chee-Leok
通讯作者：
Goh, Chee-Leok
影响因子：
3.6
作者：
Madarasingha, N. P.;Satgurunathan, K.;de Silva, M. V. C.
通讯作者：
de Silva, M. V. C.
影响因子：
4.1
作者：
Ho, M. S. L.;Ho, J.;Tan, S. H.
通讯作者：
Tan, S. H.
影响因子：
3.1
作者：
Ozcan, A;Senol, M;Karaca, S
通讯作者：
Karaca, S
影响因子：
1.1
作者：
Fernandez-Flores, Angel;Valerdiz, Saul;Rodriguez-Cernuda, Purificacion
通讯作者：
Rodriguez-Cernuda, Purificacion