cutaneous vasculits and its clinical features
cutaneous vasculits and its clinical features
Palpable purpura is the hallmark of cutaneous vasculitis but, depending on the type and degree ofvessel wall damage, other lesions, not all of which are purpuric,may occur; these include livedo reticularis, weals,papules, pustules, infarcts and ulcers.
Aetiological factors of cutaneous vasculitis include: Bacteria, e.g. streptococci, gonococci, Mycobacteriumtuberculosis (erythema induratum) and M. leprae (erythemanodosum leprosum) Viruses, e.g. hepatitis B Neoplasia, e.g. lymphoma Drugs, e.g. sulphonamides,thiazides,captopril Food additives, e.g. tartrazine Autoimmune diseases, e.g. lupus erythematosus, polyarteritisnodosa, scleroderma.The initial event is damage to the endothelium of thevessel. Both circulating and resident extravascular inflammatorycells are then activated to release mediators, someof which have destructive effects whereas others contributeto repair. The net result depends on many factors;these include the nature of the initial insult, the type andlocation of the vessels affected, and the adequacy of thecollateral blood supply. One of the most common histologicalappearances is the accumulation of neutrophils,their breakdown products, and nuclear debris arounddamaged venules, an appearance termed leukocytoclasticvasculitis. Sometimes vasculitis occurs because there isinadequate clearance of a potential cause, such as circulatingimmune complexes, from the circulation, or there is adeficiency in repair mechanisms, e.g. removal of fibrin istoo slow to maintain patency of affected vessels. A defectin the reticuloendothelial system may account for the occurrence of vasculitis in lymphomas, other malignancies and sarcoidosis. Sometimes the localization of vasculiticlesions can be explained by circumstances that slow bloodflow, e.g. in the lower limbs and where skin is cooled byoverlying fat.As well as attempting to find the cause, it is clinicallyimportant to know whether other organs are being affected, especially the brain, heart, kidneys, lungs and gut.There is no entirely satisfactory classification of multisystem vasculitis.
Clinical features Henoch-Schonlein purpuraHenoch-Schonlein purpura comprises arthralgia, abdominal pain and vasculitic rash, often with renal involvement. Children are predominantly affected. Astreptococcal sore throat and upper respiratory virus infections are the most common recognizable causes. The rashis mainly on the buttocks and extensor surfaces of thelimbs. As well as purpura there are usually erythematous macules and papules, and urticarial weals. The lesionsare characterized by a leukocytoclastic vasculitis, and inmany cases there is deposition of IgA around venules.There is no specific treatment, but some authoritiesuse high-dose corticosteroids if there is severe renaldisease.
Polyarteritis nodosa Polyarteritis nodosa, or PAN, is a multisystemdisorder in which there is necrotizing vasculitis ofsmall and medium-sized arteries. The distinctive featurein the skin is the occurrence of nodules along the courseof subcutaneous arteries, best felt on the lower limb. Inaddition to nodules there are often purpuric papules, weals,or plaques of gangrene. Livedo reticularis, a net-like arrangement of bluish venules, is common.
Malignant atrophic papulosis Malignant atrophic papulosis (Degos' disease) is a rare butvery distinctive condition in which insignificant-lookingred papules become slowly necrotic with a greyish-whitecentral scale, and heal with porcelain-like white scars.Similar lesions occur internally, particularly in the intestine,and much of the high mortality is due to perforationor haemorrhage. The brain and kidney may also beaffected.Rheumatoid diseaseSmall purple or black spots around the nailfolds are verycharacteristic of rheumatoid disease, althoughthey are also seen in other vasculitic diseases.
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