Image on Clinical Medicine: Capillary Telangiectasia in Juvenile Dermatomyositis

臨床影像判讀：毛細微血管擴張症於青少年型皮肌炎

陳士哲(Shi-Zhe Chen) ； 吳建璋(Chien-Chang Wu) ； 郭建宏(Chien-Hung Kuo) ； 黃素雲(Su-Yun Huang) ； 彭洸萍(Guang-Ping Peng) ； 高佳慧(Jia-Hui Kao) ； 段彭年(Pong- Nien Tuan)

《童綜合醫學雜誌》 13卷2期 (2019/12) Pp. 116-118

https://doi.org/10.3966/207135922019121302010

摘要

Juvenile dermatomyositis (JDM) is the early-onset form of dermatomyositis, a systemic, autoimmune inflammatory muscle disorder characterized by proximal muscle weakness, characteristic skin lesions, and systemic manifestations. The course of JDM is highly variable: Some patients go into remission within 2-3 years, whereas others followed a cyclic course marked by relapses. The aim of treatment is to reduce long-term morbidity and restore physical function. High-dose corticosteroids are the mainstay of treatment, and dose tapering after a few weeks of therapy depends on the patient response. In this imaging report, we present the case of a 10-year-old girl diagnosed with JDM based on the classical findings of heliotrope rash, Gottron's papules, poikilodermatomyositis, elevated skeletal muscle enzyme levels, bilateral proximal muscle weakness, and denervation myopathy of the left rectus femoris and biceps muscles. Additionally, periungual erythema and telangiectases, filamentous or spider-like capillary changes were observed on bilateral hands by nailfold capillaroscopy.

關鍵字

juvenile dermatomyositis ； periungual telangiectasias ； poikiloderma

並列摘要

青少年型皮肌炎，是一種系統系的自體免疫肌肉發炎疾病，以近端肢體無力以及特殊的皮膚病灶為主要表現，是皮肌炎的一種早發形式。其病程進展可長可短，可以在二至三年內病情緩減，亦可能週期性反覆發作。此疾病的治療目標首重減少長期發病率以及重建生理功能。治療初期仍以高劑量類固醇作為治療主流，之後視病人反應而做濃度調整。在這個影像報告中，該名確診為青少年型皮肌炎十歲女性，除依據典型皮膚表現，包括淡紫色皮疹、Gottron氏丘疹、肌肉酵素的增加，以及左股直肌和左股二頭肌的去神經肌病變。而其雙側手指甲床及甲周變化，更可發現微血管呈現絲狀或蛛網狀的毛細血管改變，壓後可見動脈性搏動。

並列關鍵字

青少年型皮肌炎 ； 甲周血管擴張症 ； 多形性皮炎