Systemic Scleroderma in Childhood: A Case Report

Abstract

Juvenile systemic scleroderma is a rare chronic multi-system connective tissue disease in childhood. Although rare in children, it is an important cause of morbidity and mortality. Juvenile systemic scleroderma is one of the most severe rheumatologic conditions diagnosed in children. In this article, we report a six-year-old boy diagnosed with scleroderma.Keywords: Childhood; juvenile systemic sclerosis; scleroderma.Scleroderma is derived from the Greek words skleros (hard or indurated), and derma (skin). 1Hippocrates first described this condition as thickened skin.1 Juvenile systemic sclerosis (JSSc) is a rare chronic multi-system connective tissue disease characterized by symmetrical thickening and hardening of the skin, associated with fibrous changes in internal organs, as well as vascular and immune system abnormalities in children aged 16 years or younger.2 Although rare, JSSc is among major causes of morbidity and occasional mortality.3 Besides, it is one of the most severe rheumatologic conditions diagnosed in children.3 In this article, we report a rare scleroderma case in a pediatric patient. CASE REPORTA six-year-old male patient, who presented with stiff hands and difficulty in lifting his fingers, was admitted to our clinic. His medical history was non-specific without any prior complaints. His current symptoms first appeared one and a half months ago with stiff finger tips spreading over to the toes two or three days before admission. No inquiry for prior history for medicine intake or being subject to toxic agents was taken.Physical examination revealed that he was in the 10-25 th percentile for weight, and 50-75 th percentile for height, with a pulse rate of 90 bpm and blood pressure of 90/60 mmHg. The stiffness at the palms and fingers in both hands were confirmed. His lips were tense, and he had difficulty in opening his mouth. Other examination results were non-specific.Hematological examination and blood biochemistry results were normal. Urin analysis result was also normal without proteinuria. The rheumatoid factor was negative; the viral serologic testing for anti-hepatitis A virus, immunoglobulin M, and immunoglobulin G were negative, anti-hepatitis B was positive (530.06 mIU/mL), anti-hepatitis C virus was negative, group agglutination and Rose Bengal tests were negative, Epstein-Barr virus was negative, anti-cytomegalovirus immunoglobulin M was negative, anti-cytomegalovirus immunoglobulin G was positive, and parvovirus was negative.The antinuclear antibodies (ANA) indicated to scleroderma with 1/320 positive thin granularity.

Sun Protection in Pediatric Patients With Rheumatic Disease

“…Sjögren’s and juvenile scleroderma, though less commonly, have also shown reports of adverse skin reactions to sunlight. 9,10…”