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Cutaneous vasculitis, connective tissue diseases, and urticaria 

Cutaneous vasculitis, connective tissue diseases, and urticaria

Cutaneous vasculitis, connective tissue diseases, and urticaria

Susan Burge

, and Graham S. Ogg



Following the consensus recommendation of the American College of Rheumatology, American Society of Nephrology and European League Against Rheumatism in 2011, the term Wegener’s granulomatosis is replaced by granulomatosis with polyangiitis (GPA).

Updated on 28 Nov 2013. The previous version of this content can be found here.
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date: 25 April 2017

Vasculitis (angiitis) denotes necrotizing inflammation of the blood vessels; occlusive vasculopathy implies vascular occlusion without significant vascular inflammation. A small-vessel cutaneous vasculitis is the most common vasculitis affecting the skin, and may be the first sign of a systemic vasculitis, but 50% of patients have no systemic disease. The clinical findings must be integrated with the results of serological, pathological, and imaging studies to reach a diagnosis.

Systemic lupus erythematosus (SLE) is diagnosed if four or more of the American College of Rheumatology revised criteria for the classification of SLE are present, either sequentially or simultaneously (Chapter 19.11.2). These include four mucocutaneous signs: malar rash, discoid rash, photosensitivity, and oral ulcers. Skin lesions are the first manifestation of SLE in 23 to 28% of patients; about 73% of patients report photosensitivity, and up to 91% develop cutaneous symptoms at some stage in the evolution of their disease.

Dermatomyositis is an uncommon multisystem autoimmune disease in which inflammatory skin changes are associated with polymyositis of skeletal muscle. The clinical spectrum ranges from pure cutaneous disease, through coexisting patterns of cutaneous/systemic disease, to isolated inflammatory polymyositis. Cutaneous involvement may precede the onset of myositis by several years, but some patients never have muscle involvement (amyotrophic dermatomyositis).

Scleroderma means thickened, fibrotic, bound-down skin. It may develop in association with a systemic connective tissue disease (systemic sclerosis) or present as a localized cutaneous problem. Localized scleroderma, unlike systemic sclerosis, is a self-limiting condition confined to the skin and subcutaneous tissue; it does not transform into systemic sclerosis. Dermatologists tend to use the term ‘morphoea’ for localized disease, while paediatricians and rheumatologists refer to the same condition as ‘scleroderma’.

Panniculitis is inflammation of the subcutaneous fat, sometimes associated with vasculitis. It presents with erythematous subcutaneous nodules, most often on the lower leg.

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