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一种伴有强烈瘙痒的鹅卵石样表现。

An intensely pruritic pebbled presentation.

作者信息

Soriano Livia Francine, McGrath Conn, Hawthorne Mark, Ekeowa-Anderson Arucha Linda

机构信息

Department of Dermatology, Barts Health NHS Trust, London, U.K.

出版信息

Dermatol Online J. 2018 Mar 7;24(2):13030/qt6tp6f9c4.

PMID:29630157
Abstract

Lichen amyloidosis (LA) is a form of primary localized cutaneous amyloidosis (PLCA) characterized by bilateral intensely itchy domed scaly hyperkeratotic papules. Lichen amyloidosis is rare and affects men more than women. It is uncommonly seen in the western world but more prevalent in Asia. These papules most typically affect the shins and occasionally the arms and torso. Lichen amyloidosis has been reported in association with autoimmune disorders and after prolonged exfoliation and friction of affected skin. We present a 40-year-old woman with LA. In LA, the characteristic histological finding is apple-green birefringence of Congo red-stained preparations observed under polarized light. However, this is not always strongly positive, as in our patient. Other findings may include eosinophilia, periodic acid-Schiff positivity, staining with thioflavin T, and metachromasia after staining with crystal violet or methyl violet. Treatment of LA is difficult and complete clinical remission is seldom achieved. Recent trials revealed beneficial outcomes with topical calcipotriol, phototherapy, acitretin, cyclophosphamide, and laser treatments. A combination of acitretin, antihistamines, topical steroids, and hydrocolloid dressings have been beneficial in our patient with LA.

摘要

苔藓样淀粉样变(LA)是原发性局限性皮肤淀粉样变(PLCA)的一种形式,其特征为双侧出现剧烈瘙痒的圆顶状鳞屑性角化过度丘疹。苔藓样淀粉样变较为罕见,男性患者多于女性。在西方世界较少见,但在亚洲更为普遍。这些丘疹最常累及小腿,偶尔也会出现在手臂和躯干。已有报道称苔藓样淀粉样变与自身免疫性疾病有关,以及在受影响皮肤长期剥脱和摩擦后出现。我们报告了一名40岁患有苔藓样淀粉样变的女性。在苔藓样淀粉样变中,特征性的组织学表现是在偏振光下观察到刚果红染色制剂呈现苹果绿双折射。然而,并非总是呈强阳性,就像我们的患者一样。其他表现可能包括嗜酸性粒细胞增多、过碘酸希夫染色阳性、硫黄素T染色以及结晶紫或甲基紫染色后的异染性。苔藓样淀粉样变的治疗困难,很少能实现完全临床缓解。最近的试验显示局部使用卡泊三醇、光疗、阿维A、环磷酰胺和激光治疗有良好效果。阿维A、抗组胺药、局部类固醇和水胶体敷料联合使用对我们这位患有苔藓样淀粉样变的患者有益。

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