Porokeratosis ptychotropica: a lesser-known variant

Porokeratosis ptychotropica: a lesser-known variant
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DOI:
10.1111/j.1365-2230.2009.03664.x
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发表时间:
2009-12-01
影响因子:
4.1
通讯作者:
Bhawan, J.
Bhawan, J.
中科院分区:
医学4区
文献类型:
--
作者:
Tallon, B.;Blumental, G.;Bhawan, J.

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我们报告一例罕见的汗孔角化症变异型汗孔角化症。可见环状肛周斑块和具有特征性组织学特征的多个角状板层,其下有真皮淀粉样物沉积。淀粉样蛋白沉积仅见于活检的斑块三角间区,这与其他皮肤淀粉样蛋白沉积障碍相一致,可能表明摩擦在此组织学发现的发病机制中发挥了作用。我们回顾了有关PP的文献,并总结了对治疗的不良反应。
We report a case of the rare porokeratosis variant porokeratosis ptychotropica ( PP). A circumferential perianal plaque and the characteristic histology of multiple cornoid lamellae with underlying dermal amyloid deposition were seen. Amyloid deposition was seen in the biopsied intertriginous area of the plaque only, which, in concordance with other cutaneous amyloid deposition disorders, may suggest a role for friction in the pathogenesis of this histological finding. We review the literature on PP and summarize the poor response seen to treatments.