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Connective tissue diseases can be classified into two groups: (1) a group of relatively rare genetic disorders affecting the primary structure of connective tissue; and (2) a number of acquired conditions where the connective tissues are the site of multiple, more or less distinct immune and inflammatory reactions.
The disease progressively spreads from the initial location, resulting in atrophy of the skin and its adnexa, as well as underlying subcutaneous structures such as connective tissue, (fat, fascia, cartilage, bones) and/or muscles of one side of the face. [4] The mouth and nose are typically deviated towards the affected side of the face. [5]
Rheumatism [2] /ˈruməˌtɪzəm/ or rheumatic disorders are conditions causing chronic, often intermittent pain affecting the joints or connective tissue. [3] Rheumatism does not designate any specific disorder, but covers at least 200 different conditions, including arthritis and "non-articular rheumatism", also known as "regional pain syndrome" or "soft tissue rheumatism".
Dermatomyositis is a form of systemic connective tissue disorder, a class of diseases that often involves autoimmune dysfunction. [12] [16] It has also been classified as an idiopathic inflammatory myopathy, along with polymyositis, necrotizing autoimmune myositis, cancer-associated myositis, and sporadic inclusion body myositis. [17]
Eosinophilic fasciitis (/ ˌ iː ə ˌ s ɪ n ə ˈ f ɪ l ɪ k ˌ f æ ʃ i ˈ aɪ t ɪ s, ˌ iː oʊ-,-ˌ f æ s i-/ [2] [3]), also known as Shulman's syndrome, [4] is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves.
There are also uncompleted versions of recognized connective tissue diseases, in which clinical and serological symptoms allow for a diagnosis but classification criteria are not met. [67] The most prevalent strategy to diagnosis in clinical practice combines serological criteria with at least three clinical criteria.
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Up to two thirds of those affected demonstrate some type of generalized connective tissue disorder. [ 24 ] [ 43 ] Marfan syndrome , Ehlers–Danlos syndrome , and autosomal dominant polycystic kidney disease are the three most common connective tissue disorders associated with sCSFLs. [ 24 ]