一名11岁女孩慢性苔藓样糠疹转变为杂色角化不全症。

The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl.

作者信息

Niemczyk U M, Zollner T M, Wolter M, Staib G, Kaufmann R

机构信息

Department of Dermatology, University of Frankfurt Medical School, Germany.

出版信息

Br J Dermatol. 1997 Dec;137(6):983-7.

PMID:9470920

Abstract

Parakeratosis variegata is a rare disorder with unknown aetiology. In a few cases it arises from benign skin diseases such as pityriasis lichenoides et varioliformis acuta (Mucha Habermann disease) or pityriasis lichenoides chronica. However, transformation into malignant diseases such as cutaneous T-cell lymphoma has been observed. We report an 11-year-old girl with a 10-year history of pityriasis lichenoides chronica now presenting with parakeratosis variegata. Analysis of skin infiltrating T cells showed clonally rearranged T-cell receptor gamma chains occurring with a frequency of more than 2%. This finding is compatible with the clinical observation of parakeratosis variegata transforming into a malignant T-cell disorder. We therefore suggest that patients suffering from parakeratosis variegata and other diseases such as pityriasis lichenoides et varioliformis acuta or pityriasis lichenoides chronica should be continuously monitored.

摘要

斑驳性角化不全是一种病因不明的罕见疾病。少数情况下，它由良性皮肤病如急性痘疮样苔藓样糠疹（穆查-哈伯曼病）或慢性苔藓样糠疹引起。然而，已经观察到其转变为恶性疾病，如皮肤T细胞淋巴瘤。我们报告一名11岁女孩，有10年慢性苔藓样糠疹病史，现出现斑驳性角化不全。对皮肤浸润性T细胞的分析显示，T细胞受体γ链发生克隆性重排，频率超过2%。这一发现与斑驳性角化不全转变为恶性T细胞疾病的临床观察结果相符。因此，我们建议对患有斑驳性角化不全以及其他疾病如急性痘疮样苔藓样糠疹或慢性苔藓样糠疹的患者进行持续监测。