Scleroderma (overview) M34.9

Diseases from the classic "fibrotic" form, whereby systemic scleroderma (systemic sclerosis) can be regarded as a prototype of multisystem sclerosis. Circumscribed localized scleroderma is a mono-organic (skin) infection. The leading symptom of scleroderma is the fibrotic remodelling (sclerosis) of the skin.

Basically one can distinguish:

• Circumscribed (or localized) scleroderma: in this case, sclerosis and atrophy are predominantly confined to the skin, the subcutaneous adipose tissue and, depending on the location, also to the underlying bone.
• Systemic scleroderma (systemic sclerosis), a multi-organ disease with a progressive, potentially fatal course, affecting numerous internal organs in addition to the skin (especially the digestive tract, lungs, vessels, heart and kidneys).

For differential diagnostic reasons, disease patterns with a scleroderma-like aspect or course must be separated, the pseudoscleroderma.

S.u. Scleroderma, circumscripts, see below Scleroderma, systemic.

1. Fabris M, Hunzelmann N (2007) Differential diagnosis of scleroderma and pseudoscleroderma. JDDG 11:977-984
2. Kayser C et al(2015) Autoantibodies in systemic sclerosis: unanswered questions. Front Immunol 6:167