Childhood disabling Pansclerotic Morphoea complicated by leg ulcers, contractures and gangrene
- Author:
Pan JY
;
Ker KJ
;
Tang MBY
- Publication Type:Case Reports
- Keywords:
morphea, ulcers, contracture, gangrene
- From:Malaysian Journal of Dermatology
2010;25(-):25-30
- CountryMalaysia
- Language:English
-
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.
