Jamalpur Indirakshi, Mogili Harikrishna Reddy, Koratala Abhilash
Department of Dermatology, Kurnool Medical College, Kurnool, Andhra Pradesh, India.
Department of Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India.
BMJ Case Rep. 2018 Feb 17;2018:bcr-2017-222132. doi: 10.1136/bcr-2017-222132.
Disabling pansclerotic morphoea (DPM) of childhood is a severe and often fatal variant of deep morphoea. It usually starts in childhood and rarely seen in adults. The course of the disease is progressive with lifelong morbidity in the form of joint contractures and immobility. The causes of mortality include complications such as sepsis, gangrene and cardiopulmonary involvement. Herein, we discuss the case of a 15-year-old girl with limb deformity and finger contractures, that is, bone involvement. The diagnosis of DPM of childhood was fortuitously made after the correction of limb deformity, when the patient was seen in the dermatology department for evaluation of skin discolouration on the thighs.
儿童致残性全硬化性硬斑病（DPM）是深部硬斑病的一种严重且常致命的变体。它通常始于儿童期，在成人中很少见。疾病进程呈进行性，会导致关节挛缩和活动障碍等终身残疾。死亡原因包括败血症、坏疽和心肺受累等并发症。在此，我们讨论一名15岁女孩的病例，她有肢体畸形和手指挛缩，即骨骼受累。该患儿在矫正肢体畸形后偶然被诊断为儿童DPM，当时她因大腿皮肤变色到皮肤科就诊。
