Idiopathic thrombocytopenic purpura itp, also known as primary immune thrombocytopenic purpura and autoimmune thrombocytopenic purpura, is defined as isolated thrombocytopenia with normal bone marrow and in the absence of other causes of thrombocytopenia. Henochschonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Plasmapheresis with plasmatic exchange is the treatment of choice. Interventions for preventing and treating kidney disease in henoch. Henochschonlein purpura italy pdf ppt case reports. Vascular purpura main etiologies encompass the wide specturm of vasculitides and microvascularocclusion syndromes.
Vascular purpura definition of vascular purpura by. Understanding the pathophysiology of thrombotic thrombocytopenic purpura is continuous and too early to have clearly defined evidencebased standards applicable to patient management and treatment. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Vascular purpura, also called henoch schonlein purpura, can range from a mild, selflimited condition to a serious illness of the kidney or intestines. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved.
Palpable purpura is purpura than can be felt, due to inflammation of the blood vessels vasculitis. Pathologically, it can be considered a form of leukocytoclastic vasculitis that can involve not only the skin but other tissues as well. Purpura or bleeding within the first month of life should be differentiated from different types of purpuric skin diseases. Easy bruising appears to run in families, and is often seen in women of childbearing age who develop discolored patches on their upper arms, thighs, and buttocks without. Hoy, en, os contamos mas sobre otra enfermedad autoinmune. With kidney involvement, there may be a loss of small amounts of blood and. It is believed to be a disorder of the immune system. Background purpura fulminans, first described by guelliot in 1884, is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation dic. Although doctors often do tests of the blood clotting system looking for clotting abnormalities, no abnormalities are found see also bruising and bleeding.
Henochschonlein purpura with bilateral central retinal. Henochschonlein purpura definition of henochschonlein. Chapter 121 the vascular purpuras free medical textbook. George department of medicine, college of medicine, department of biostatistics and epidemiology, college of public health, university of oklahoma health sciences center, oklahoma city, ok, usa. The initial presentation is usually with purpura and joint pain 20. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain. Visit our research pages for current research about purpura treatments clinical trials for purpura. Thrombotic thrombocytopenic purpura linkedin slideshare.
Petechiae are small, purpuric lesions up to 2mm across. Chapter 121 the vascular purpuras williams hematology chapter 121 the vascular purpuras paul i. Itp has two distinct clinical syndromes, manifesting as an acute condition in children. Cerebral vasculitis in henochschonlein purpura article pdf available in nephrology dialysis transplantation 152. Purpura fulminans pf is a haemorrhagic condition usually associated with sepsis or previous infection. Then progress petechiae or purpura which are characterized clinically as palpable pupura that evolve from red to purple rusty brown before they eventually fade.
The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. Schneiderman nonpalpable purpura increased transmural pressure gradient conditions affecting the integrity of the blood vessel wall and its supporting tissues trauma to blood vessels palpable purpura cutaneous vasculitis dysproteinemias primary cutaneous diseases. Bruises develop on the thighs, buttocks, and upper arms in people without known injuries. Volume 35, issue 1, januaryfebruary 2017, pages 6880. It makes blood clot in the small blood vessels all over the body. Ecchymoses or bruises are larger extravasations of blood. Immunoglobulin a nephropathy and iga vasculitis henochschonlein purpura. Hemorrhagic disease of the newborn is due to an accentuation of the normal fall of prothrombin within the first week of life. Vasculitis has many types, each with different symptoms. Henoch schonlein purpura is a small vessel vasculitis that affects mainly. Purpura occurs when small blood vessels leak blood under the skin. Similar lesions are produced in many specific diseases, e. It can occur at any age, but usually affects children.
A greater focus on thrombotic thrombocytopenic purpura has emerged in recent years with advances in pathophysiology and diagnostic testing. This syndrome usually occurs in children, but it has also been noted in a. These small clots can block the flow of blood to major organs, such as the kidneys, brain, or heart. Purpura are nonblanchable, hemorrhagic skin lesions that result from the leakage of red blood cells into the skin. Purpura are non blanching red or purple spots caused by bleeding beneath the skin. Hsp is most common in younger pts clinical red maculopapules on legs and buttocks. Purpura measure between 4 and 10 mm millimeters in diameter. Vascular purpura revealing a severe dilated cardiomyopathy. In contrast to other erythematous or vascular skin lesions, purpuric lesions do not blanch when pressure is applied to the skin. Paciente con purpura cutanea monica rodriguez carballeira. These clinical manifestations may develop over the course of days to weeks. Wecome to the official fb page of the ttp association. Thrombotic thrombocytopenic purpura association home. As with iga nephropathy, serum levels of iga are high in hsp and there are identical findings on renal biopsy.
The spots are caused by bleeding underneath the skin secondary to platelet disorders, vascular disorders, coagulation disorders, or other causes. Thrombotic thrombocytopenic purpura ttp was first described by moschowitz in 1924. People may have family members who also bruise easily. She has torpid evolution, and gastrointes tinal and renal complications. The clinical implications of adultonset henochschonelin.
Vascular purpura is uncommon in the neonatal period but may occur. Signs of vasculitis include red spots purpura, lumps nodules or sores ulcers on the skin. Retiform purpura can occur in a variety of disorders. Henochschonlein purpura is a small vessel vacuities in which complexes of immunoglobulin a iga and complement component 3 c3 are deposited on arterioles, capillaries, and venules. Thrombotic thrombocytopenic purpura ttp is a disease with a high rate of mortality if a proper treatment is not instated. Definition, diagnosis and treatment of immune thrombocytopenic purpura james n. Hsp cinical manifestations the lesions tend to occur in crops lasting from 310 days and may appear at. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Henoch schonlein purpura hsp is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. Careful evaluation of any patient with purpura is important for early diagnosis and treatment, particularly in.
Purpura trombocitopenica idiopatica by adrian flores on. Henochschonlein purpura united states pdf ppt case. Features include tissue necrosis, small vessel thrombosis and disseminated intravascular coagulation. Sus manifestaciones clinicas mas frecuentes son bien cono. Among them, cardiac embolism represents an unusal but potentially severe etology.
Vascular purpura, also called henoch schonlein purpura, can range from a mild, self. Purpura is purplecolored spots and patches that occur on the skin, and in mucus membranes, including the lining of the mouth. Vascular dermatoses purpura and vasculitis author links open overlay panel jana kazandjieva md, phd a dimitar antonov md, phd b jivko kamarashev md, phd c nikolai tsankov md, phd. Purpura symptoms, diagnosis, treatments and causes. We present a case of vascular purpura revealing an intracardiac leftsided thrombus complicating an endstage dilated cardiomyopathy. Vasculitis may seriously affect kidneys but have no. Of the various types of vascular bleeding, two of the most common are purpura simplex, a condition marked by easy bruising, and senile purpura, which occurs among older people.
806 1338 1239 564 45 364 950 561 1286 470 1518 319 254 355 1576 1081 839 20 804 1045 612 236 1371 782 1423 1403 1093 1387 103 34 357 1510 742 804 1522 1325 1417 387 1488 416 1322 751 1271 1217 1026 1429 1258 210 144 831