Childhood disabling Pansclerotic Morphoea complicated by leg ulcers, contractures and gangrene

ABSTRACT

Disabling pansclerotic morphoea of childhood is a subset of localized scleroderma. It is a rare disease in both the adult and paediatric population. E t i o l ogical factors are unknown although autoimmune, infectious, genetic and environmental factors have been postulated. Sclerotic plaques predominantly affect the scalp, face, trunk and extensor surfaces of limbs, leaving fingertips and toes uninvo l ved. The absence of Raynaud’s phenomenon, dysphagia, visceral involvement and certain laboratory derangements diff e r e n t i a t e systemic sclerosis and disabling pansclerotic morphoea of childhood. Diagnosis can be supported by histology. There are seve r a l management options including topical, systemic and phototherapy.