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The so-called connective tissue diseases are the result of environmental stimuli (such as drugs, infection or exposure to ultraviolet radiation) in genetically predisposed individuals. They are associated with a range of circulating autoantibodies.
Systemic lupus (SLE) is characterised by:
Drug-induced lupus may be due to a variety of antibiotics (in adolescents, most often minocycline), antihypertensive and anti-inflammatory medications.
Dermatopathological findings in SLE include a lichenoid tissue reaction and a band of immunoglobulins at the dermo-epidermal junction (detected by direct immunofluorescence of clinically involved skin).
Investigations should include:
Scleroderma (tight skin) is seen in the following conditions:
Morphoea is characterised by ivory white oval sclerotic plaques with an inflamed edge that eventually result in post-inflammatory pigmentation and dermal or subcutaneous atrophy. It is not uncommon in children and adolescents.
A linear variety can involve underlying muscle and bone and is more common in children. If it affects the face and /or scalp, it is also known as en coup de sabre.
Systemic sclerosis is characterised by progressive accumulation of collagen, fibrosis and loss of mobility of the skin and other organs such as the respiratory and gastrointestinal tracts. it is exceedingly rare in children
The CREST variant (Calcinosis, Raynaud's, oEsophageal, Sclerodactyly, Telangiectasia) may have less severe systemic manifestations.
Treatment is mainly symptomatic at this time.
Chronic graft-versus-host disease (GVHD)
Systemic-onset juvenile rheumatoid arthritis is sometimes associated with an evanescent salmon-pink rash on the trunk. Rheumatoid nodules, vasculitis and leg ulcers are more common in adults with rheumatoid disease.
Describe the management of cutaneous lupus erythematosus in pregnancy.
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