Abstract
Juvenile localized scleroderma (JLS) is a rare chronic inflammatory and fibrosing disorder. It can result in significant morbidity, disfigurement, and severe functional, aesthetic and psychological disabilities. Patients with JLS should be identified early, evaluated extensively, treated aggressively, and monitored carefully. Here the case of a 2 year old boy is reported who was admitted into the department of Paediatrics of Delta Medical College & Hospital, Dhaka, Bangladesh with painful swelling of all fingers of both hands for 6 months and blackish patches over the fingers for the last one and half months. Left little finger was the first finger affected and there was flexion contracture of both left little and index fingers. The boy was diagnosed as a case of juvenile localized scleroderma and was confirmed by skin biopsy. We treated the child with methotrexate and prednisolone. It is very essential to raise awareness about this disease among clinicians and also parents for early diagnosis and treatment.Delta Med Col J. Jul 2015; 3(2): 96-101
Highlights
Scleroderma is a chronic systemic autoimmune disease characterized by hardening of the skin
Scleroderma is characterized by increased synthesis of collagen, damage to small blood vessels, activation of T lymphocytes and production of altered connective tissues.[3]
Known as ‘morphea’, has a different pattern of skin involvement than systemic sclerosis (SSc) and encompasses several subtypes classified by depth and pattern of the lesion(s)
Summary
Scleroderma is a chronic systemic autoimmune disease characterized by hardening (sclero) of the skin (derma). Upto 25% of children with LS have extracutaneous manifestations most commonly arthritis (47%) and neurologic symptoms (17%) associated with en coup de sabre.[3] Localized scleroderma, known as ‘morphea’, has a different pattern of skin involvement than systemic sclerosis (SSc) and encompasses several subtypes classified by depth and pattern of the lesion(s). A 2 years old male child hailing from Mohakhali, Dhaka was admitted into the department of Paediatrics of Delta Medical College & Hospital on 25-2-2014 with painful swelling of all fingers of both hands for 6 months and blackish patches over the fingers for the last one and half months. There was no history suggestive of associated endocrine problem He was the second issue of his parents and his parents were first degree cousins. The child was diagnosed as a case of localized scleroderma deep morphea type with nutritional anaemia, protein energy malnutrition and haemoglobin E trait. Follow up laboratory findings were suggestive of control of disease (e.g. eosinophil count 2%)
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