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Gottron's sign is a pathognomonic cutaneous manifestation associated with dermatomyositis (DM), which is an inflammatory disorder affecting the skin and muscles. [1] The primary lesion of dermatomyositis appears as a violaceous, macular erythema with a symmetric distribution, which may progress and become poikilodermatous (atrophic with telangiectasia and pigmentary changes) and indurated (as ...
Dermatomyositis is an autoimmune disorder featuring both humoral and T-cell autoimmune processes. [3] Dermatomyositis may develop as a paraneoplastic syndrome associated with several forms of malignancy. [4] It is known to be associated with several viruses, especially coxsackievirus, but no definitive causal link has been found. [3]
The three main types of idiopathic myositis (known as inflammatory myopathies) that typically test positive for autoantibodies are dermatomyositis, polymyositis, and inclusion body myositis. [4] Other autoimmune diseases, such as systemic lupus erythematosus , can also cause myositis-like symptoms.
It can also be associated with underlying cancer. The main classes of idiopathic inflammatory myopathy are polymyositis (PM), dermatomyositis (DM) (including juvenile, amyopathic, and sine-dermatitis form), inclusion-body myositis (IBM), immune-mediated necrotising myopathy (IMNM), and focal autoimmune myositis. [1]
Gowers's sign is classically seen in Duchenne muscular dystrophy where it is mostly evident at 4–6 years, but also presents itself in centronuclear myopathy, myotonic dystrophy and various other conditions associated with proximal muscle weakness, including Becker muscular dystrophy, dermatomyositis and Pompe disease.
When sIBM was originally described, the major feature noted was muscle inflammation. Two other disorders were also known to display muscle inflammation, and sIBM was classified along with them. They are dermatomyositis (DM) and polymyositis (PM) and all three illnesses were called idiopathic (of unknown origin) myositis or inflammatory ...