青少年皮肌炎(JDM)是一种慢性自身免疫性炎症性疾病,被认为是特发性炎症性肌病的最常见形式。JDM主要影响皮肤和骨骼肌。特征性体征和症状包括Gottron丘疹,天草性皮疹,角质层钙质沉着,和对称的近端肌肉无力。然而,JDM表现为广义鳞状鳞茎皮病是一种陌生的表现。在此,我们报告了一名14个月大的女性幼儿,从她七个月大的时候开始,出现了广泛的进行性无症状鳞状斑驳的色斑(多皮病)。她的实验室结果并不显著。她被诊断为多皮性皮疹,并与肌病性皮肌炎进行了鉴别诊断,多皮病,和斑贴期真菌病。只给她开了保湿霜。一年后,在后续行动中,她展示了JDM的全貌,随着鳞片状皮肤斑块的历史变得更加普遍,口服期间经常窒息,不能站着坐着。实验室检查对于低白细胞和血红蛋白计数很重要,随着CPK的升高,LDH,铁蛋白,CRP,和ESR水平。MRI显示右前大腿和股外侧皮下水肿。因此,该患儿被诊断为JDM.
Juvenile dermatomyositis (JDM) is a chronic autoimmune inflammatory disorder and is considered the most common form of idiopathic inflammatory myopathies. JDM primarily affects the skin and the skeletal muscles. Characteristic signs and symptoms include Gottron papules, heliotrope rash, calcinosis cutis, and symmetrical proximal muscle weakness. However, JDM presenting with generalized scaly poikeloderma is an unfamiliar presentation. Herein we report a 14-month-old female toddler presented with generalized progressive asymptomatic scaly mottled violaceous patches (poikilodermatous) that started when she was seven months old. Her lab results were unremarkable. She was diagnosed with poikilodermatous skin rash with a differential diagnosis of Amyopathic dermatomyositis, poikilodermatous genodermatosis, and patch-stage mycosis fungoides. She was prescribed moisturizer creams only. A year later, during a follow-up, she presented with a full picture of JDM, with a history of scaly poikilodermatous skin patches that became more widespread, frequent choking during oral intake, and not being able to stand and sit unsupported. Laboratory workup was significant for low WBC and hemoglobin counts, along with elevated CPK, LDH, ferritin, CRP, and ESR levels. MRI revealed the right anterior thigh and vastus lateralis subcutaneous edema. Therefore, the child was diagnosed and treated as a case of JDM.