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与系统性肥大细胞增多症相关的掌跖角化病:转化生长因子-α的作用及干扰素-α的疗效

Tripe palms associated with systemic mastocytosis: the role of transforming growth factor-alpha and efficacy of interferon-alfa.

作者信息

Chosidow O, Bécherel P A, Piette J C, Arock M, Debré P, Francès C

机构信息

Department of Internal Medicine, Université Paris 6, Groupe Hospitalier Pitié-Salpêtrière, France.

出版信息

Br J Dermatol. 1998 Apr;138(4):698-703. doi: 10.1046/j.1365-2133.1998.02190.x.

DOI:10.1046/j.1365-2133.1998.02190.x
PMID:9640384
Abstract

Tripe palms are thickened, moss-like or velvety textured exaggerations of the normal dermatoglyphics. The disease belongs to the spectrum of papulosquamous paraneoplastic syndromes. Although suspected, the role of transforming growth factor-alpha (TGF-alpha) has not been clearly established. A 54-year-old man with systemic mastocytosis presented with thickening and darkening of the palms and soles. We performed skin biopsies for light microscopy (including toluidine blue), in situ hybridization and double labelling, and determination of serum tryptase, histamine and TGF-alpha levels. Toluidine blue stained the mast cells that had massively infiltrated the dermis. Tripe palm samples showed extensive hyperkeratosis. The TGF-alpha probe reacted strongly with the mast cells that also reacted with the antitryptase monoclonal antibody. Elevated tryptase, histamine and TGF-alpha levels prior to interferon-alfa administration decreased under treatment. The demonstration of TGF-alpha in infiltrating mast cells, the clinical regression of tripe palms and the lowering of the serum level and the mast cell molecular signal of the cytokine when systemic mastocytosis was controlled by interferon-alfa, suggest a key role for TGF-alpha in this cutaneous paraneoplastic syndrome.

摘要

掌跖角化过度症表现为正常皮纹增厚,呈苔藓样或天鹅绒样质地。该疾病属于丘疹鳞屑性副肿瘤综合征范畴。尽管有人怀疑转化生长因子-α(TGF-α)的作用,但尚未明确证实。一名54岁的系统性肥大细胞增多症男性患者出现手掌和脚底增厚及色素沉着。我们进行了皮肤活检,包括光镜检查(含甲苯胺蓝染色)、原位杂交和双重标记,并测定血清类胰蛋白酶、组胺和TGF-α水平。甲苯胺蓝染色显示大量肥大细胞浸润真皮。掌跖角化过度症样本显示广泛的角化过度。TGF-α探针与也与抗类胰蛋白酶单克隆抗体反应的肥大细胞强烈反应。在给予干扰素-α治疗前,类胰蛋白酶、组胺和TGF-α水平升高,治疗后降低。在浸润的肥大细胞中发现TGF-α,掌跖角化过度症临床症状消退,以及当系统性肥大细胞增多症由干扰素-α控制时细胞因子血清水平和肥大细胞分子信号降低,提示TGF-α在这种皮肤副肿瘤综合征中起关键作用。

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Tripe palms associated with systemic mastocytosis: the role of transforming growth factor-alpha and efficacy of interferon-alfa.与系统性肥大细胞增多症相关的掌跖角化病:转化生长因子-α的作用及干扰素-α的疗效
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