Sclerodermie, een teken van Veroudering

Hutchinson-Gilford progeria syndrome (HGPS) is regarded as a model for aging. Moreover, the mutant protein accumulating in progeria is to some extent also found in healthy aged individuals. Patients with HGPS show sclerotic plaques and patients with the neonatal lethal variant of progeria, i.e. restrictive dermopathy, show a generalized taut and tense skin. These aging prototypes suggest that scleroderma is related to aging. Scleroderma is also the major characteristic of systemic sclerosis, a devastating autoimmune disease characterized by vascular and immune dysfunction as well as evident fibrosis affecting skin and internal organs. It is classified according to skin involvement into a limited variant, a diffuse variant and systemic sclerosis sine scleroderma. Importantly, regardless of the subtype, systemic attrition can be seen in all of these. The symmetrical distribution of scleroderma starting at the hands and evolving more proximally is the most characteristic feature. Also the presence of Raynaud's phenomenon and abnormal capillaroscopy findings including giant capillaries, hemorrhage and loss of capillaries aid to diagnose systemic sclerosis.