Mechanic’s Hands and Hiker’s Feet in a Child with Juvenile Dermatomyositis Overlap Syndrome

IF 0.2 Q4 DERMATOLOGY
T. Vasanthi, Ramkumar Ramamoorthy, Mahesh Janarthanan
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引用次数: 0

Abstract

A 10-year-old boy presented with a history of rashes on his palms and soles for 1 year. He had difficulty in walking, climbing stairs, and worsening of skin lesions for 2 months. On examination, he had hyperkeratotic, scaly lesions with fissures on his hands and feet suggestive of mechanic’s hands [Figure 1a] and hiker’s feet [Figure 1b]. Fingernails appeared dystrophic with pitting [Figure 2a]. Musculoskeletal examination showed severe proximal muscle weakness with a positive Gower’s sign. His investigations revealed Hemoglobin (Hb) – 13.4 g/dl (12–15), white blood cell count – 13,300/cumm (4000–11,000), neutrophil – 61% (45–70), lymphocyte – 28% (20–40), platelets – 3.65/cumm (1.5 to 4.5), erythrocyte sedimentation rate (ESR) – 42 mm/h (4-–12), C-reactive protein (CRP) – 2 mg/L (<5), aspartate transaminase (AST) – 435 U/L (8-48), alanine transaminase (ALT) – 1753(7-55), creatine phosphokinase (CPK) – 5336 IU/L (20-200), and lactate dehydrogenase (LDH) – 1536 U/L (110-295); renal functions were normal. Antinuclear antibody (ANA) was positive by indirect immunofluorescence method 1:320, 3 +. Antibody screen for myositis profile revealed strong positivity for PM-Scl antibodies. Magnetic resonance imaging of the thighs revealed altered signal intensity within muscles suggestive of inflammatory myositis and high-resolution computed tomography of the chest revealed fibrosis in the anterior segment of the right middle lobe and posterior basal segment of the right lower lobe. He was diagnosed with dermatomyositis overlap syndrome and commenced on steroids, monthly intravenous (IV) cyclophosphamide, weekly subcutaneous methotrexate, and sunscreen. With treatment, there was improvement in muscle strength, complete resolution of skin changes; with nails initially showing demarcation of area of normal growth from the dystrophic part [Figure 2b] and later completely normal nails [Figure 2c].Figure 1: (a) Mechanic’s hands, (b) Hiker’s feetFigure 2: (a) dystrophic nails with pitting, (b) post treatment resolution of skin changes and nails showing demarcation between area of normal growth and dystrophic part, (c) complete resolution of skin and nail changesThe term mechanic’s hands is used to describe hyperkeratotic lesions that maybe present in the hands and fingers of patients with inflammatory myositis.[1] Equivalent lesions present in the feet were later termed hiker’s feet.[2] These findings are mostly associated with the presence of anti-Jo-1 synthetase antibody and rarely in dermatomyositis overlap syndromes.[3] The disease course tends to be chronic and may require the administration of high-dose steroids, followed by a taper, disease-modifying agents, and/or rituximab. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
少年皮肌炎重叠综合征儿童的机械师手和徒步旅行者脚
一个10岁的男孩提出了他的手掌和脚底皮疹的历史1年。行走、爬楼梯困难,皮肤病变加重2个月。经检查,他的手和脚有角化过度,鳞状病变和裂缝,提示技工的手[图1a]和徒步旅行者的脚[图1b]。指甲出现营养不良,有麻点[图2a]。肌肉骨骼检查显示严重的近端肌无力,伴有高尔氏征阳性。他的调查显示血红蛋白(Hb) - 13.4 g/dl(12 - 15),白细胞计数- 13,300/cumm(4000-11,000),中性粒细胞- 61%(45-70),淋巴细胞- 28%(20-40),血小板- 3.65/cumm(1.5至4.5),红细胞沉降率(ESR) - 42 mm/h (4- 12), c反应蛋白(CRP) -2 mg/L(<5),天冬氨酸转氨酶(AST) - 435 U/L(8-48),丙氨酸转氨酶(ALT) - 1753(7-55),肌酸磷酸激酶(CPK) - 5336 IU/L(20-200),乳酸脱氢酶(LDH) - 1536 U/L (110-295);肾功能正常。间接免疫荧光法1:32,3 +检测抗核抗体(ANA)阳性。肌炎抗体筛选显示PM-Scl抗体强阳性。大腿磁共振成像显示肌肉内信号强度改变提示炎症性肌炎,胸部高分辨率计算机断层扫描显示右中叶前段和右下叶后基段纤维化。他被诊断为皮肌炎重叠综合征,并开始使用类固醇,每月静脉注射(IV)环磷酰胺,每周皮下注射甲氨蝶呤和防晒霜。经过治疗,肌肉力量得到改善,皮肤变化完全消失;指甲最初显示正常生长区域与营养不良部分的界限[图2b],后来指甲完全正常[图2c]。图1:(a)机械师的手,(b)登山者的脚图2:(a)带点蚀的营养不良指甲,(b)治疗后皮肤变化和指甲的分辨率显示正常生长区域和营养不良部分之间的界限,(c)皮肤和指甲变化的完全分辨率机械师的手这个术语用于描述炎症性肌炎患者的手和手指可能出现的角化过度病变。[1]脚上出现的类似病变后来被称为徒步旅行者的脚。[2]这些发现大多与抗jo -1合成酶抗体的存在有关,很少出现在皮肌炎重叠综合征中。[3]病程往往是慢性的,可能需要给药大剂量类固醇,随后逐渐减少,疾病调节剂,和/或利妥昔单抗。作者证明他们已经获得了所有适当的同意书,并由患者的父母/监护人正式签署。在此表格中,家长/监护人已经/已经同意其孩子的图像和其他临床信息将在杂志上报道。家长明白他们孩子的名字和首字母不会被公布,并会尽力隐藏他们的身份,但不能保证匿名。财政支持及赞助无。利益冲突没有利益冲突。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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