Rapidly progressing juvenile scleroderma: A case report

Dr Dhanya Soodhana Mohan,Dr Zalak Shah

doi:10.17511/ijpr.2018.i06.07

Abstract

Juvenile Scleroderma encompasses a range of conditions unified by the presence of fibrosis of the skin. Juvenile scleroderma is of two major types: Juvenile localized scleroderma and Juvenile Systemic sclerosis. Juvenile localized scleroderma is the predominant type seen in children (>95%). We report a case of systemic sclerosis in a child, which is associated with higher mortality and morbidity. Apart from the characteristic mask like face, arthritis was the predominant complaint in our case.

Highlights

The term scleroderma literally means sclerosing or hardening of the skin
The various organ systems involved in juvenile Systemic sclerosis (SSc) are skin, GIT, respiratory, renal, musculoskeletal and cardiovascular
Confirming the diagnosis of Systemic Sclerosis, the patient was started on a weekly regimen of methotrexate, 15 mg/m2as a single oral dose as a therapy for the skin involvement.The child was given pulse methyl prednisolone therapy for 3 days a month for three months

Summary

Introduction

“Scleroderma” encompasses both forms of the disease: Systemic sclerosis (SSc), is characterized by the fibrosis of the skin, vascular and visceral organs and is more common among the adults and localized scleroderma (LS), is characterized by fibrosis of skin and underlying tissue without vascular or internal organ involvement, is more common amongst children They both share a common underlying pathophysiology of excessive collagen deposition in an autoimmune setting. The child was apparently normal 6 months back when she started developing pain in the joints starting with the joints of the hand, wrist and elbow which later involved the ankle and the knee joints. Confirming the diagnosis of Systemic Sclerosis, the patient was started on a weekly regimen of methotrexate, 15 mg/m2as a single oral dose as a therapy for the skin involvement.The child was given pulse methyl prednisolone therapy for 3 days a month for three months. The parents were told to avoid cold, trauma, heat and sun exposure as cold and trauma could exacerbate the symptoms

Discussion

Conclusion