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Linear scleroderma as one of the manifestations of collagenosis

Objectives: Systemic scleroderma is an autoimmune disease, which is based on generalized microangiopathy and activation of fibrosis processes in the skin and internal organs. It is characterized by immunological disorders, vascular damage and increased accumulation of extracellular matrix proteins in the skin. Purpose: to make differential diagnosis and show an example of a patient with a linear form of scleroderma. Methods: We examined 12 y.o. girl, who came to us with complaints of limb slimming of the forearm and hand and shortening of 4-5 fingers. To make a correct diagnosis and to exclude such diseases, as duchenne-Erb palsy, cerebral palsy, neuropathy of ulnar nerve were investigated the neurological examination, MRI, EMG and ENMG. Results: Neurological examination: hypotrophy on the right hand, muscle hypotonia and tendon reflexes were high on both sides, sealing on the skin along the ulnar nerve, smooth, dense consistency. There were no contractures. Sensitivity was normal. Hands movements were not limited. MRI of the brain: residual encephalopathy. Surface EMG: was registered type IIB - III EMG according to Yussevich, which indicates signs of combined cerebro-spinal pathology. Stimulation EMG: amplitude and speed indicators of the M response in the motor fibers of the ulnar, radial and median nerves on the right were within the normal range. Conclusions: Based on the patient's complaints, neurological examination, neuroimaging, was made a diagnosis of systemic scleroderma and the patient was referred for a consultation and treatment to a rheumatologist.
Keywords: Systemic scleroderma, duchenne-Erb palsy, cerebral palsy, neuropathy of ulnar nerve.

Sarafroz Fayzullaeva
City Children's Consultative and Diagnostic Center
Uzbekistan

Nilufar Ziyamukhamedova
Center for the development of professional qualification of medical workers
Uzbekistan

 

 


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