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A Unique Association of Unilateral Idiopathic Calcinosis Cutis with Ipsilateral Porokeratotic Eccrine Ostial and Dermal Duct Nevus

机译:单侧特发性Calcinosis角质层与同侧Porokeratotic内分泌和真皮管痣的独特联系

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摘要

An 11-year-old boy presented with complaints of multiple skin-colored hard lumps on the right side of his body and progressive deformity of the right leg of 7-years duration. His parents had also noticed multiple asymptomatic pits over his right arm, palms, and soles since childhood. Examination revealed skin-colored nontender nodules on the right half of his body and shortening of his right leg. The multiple hyperpigmented pits over the right arm, palm, and sole raised diagnostic difficulties, but histopathologic, radiologic, and biochemical investigations confirmed the features of idiopathic calcinosis cutis and porokeratotic eccrine ostial and dermal duct nevus. Unilateral idiopathic calcinosis cutis has not been previously reported in the literature, and the association with ipsilateral porokeratotic eccrine ostial and dermal duct nevus makes this case unique. Diagnostic difficulties and limited options for treatment make this case interesting academically.
机译:一个11岁的男孩抱怨身体右侧出现多个肤色硬块,并持续7年,导致右腿进行性畸形。他的父母还从小就注意到他的右臂,手掌和脚掌上有多个无症状的凹陷。检查发现他的身体右半部分皮肤上呈浅色结节,右腿缩短。右臂,手掌和足底上有多个色素沉着的凹坑,增加了诊断难度,但是组织病理学,放射学和生化检查证实了特发性皮肤角质病和角化性角膜外分泌性猫眼和真皮管痣的特征。先前尚未有文献报道单侧特发性角质层皮肤角质病,而与同侧猪角化性内分泌小孔和真皮管痣的相关性使该病例独特。诊断上的困难和有限的治疗选择使这种情况在学术上变得有趣。

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