Scleroderma: the hard skin

The term scleroderma covers a heterogeneous group of diseases sharing the characteristic feature of cutaneous sclerosis. The individual diseases vary in the involvement of affected structures such as skin, adipose tissue, muscles, joint structures, bones, internal organs and the associated disease expression. Basically, two different subtypes can be distinguished: localised scleroderma (LoS) and systemic sclerosis (SSc), each of which are subdivided into further subgroups. These are two distinct diseases that differ in the extent of skin sclerosis, involvement of internal organs, antibody profile, disease management and prognosis. In addition, there are clinical pictures that mimic scleroderma and are therefore referred to as scleroderma-like diseases or pseudoscleroderma. In order to make the correct diagnosis, a standardised diagnostic procedure is significant. Early diagnosis is important in order to initiate early treatment and thus minimise functional cutaneous and extracutaneous impairment as well as cosmetic damage