11/72. Papular-purpuric "gloves and socks" syndrome due to parvovirus B19: report of a case with unusual features.We present a case of papular-purpuric "gloves and socks" syndrome (PPGSS) in an adult male with acute parvovirus B19 infection. The patient displayed the classical features of fever, oral lesions, and purpura on hands and feet, but the purpuric lesions on the feet evolved to superficial skin necrosis, a feature not previously described in this syndrome. We believe this is the first reported case of PPGSS occurring in brazil.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
12/72. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura.Upshaw-Schulman syndrome (USS) is a congenital bleeding disorder characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma. Inheritance of USS has been thought to be autosomal recessive, because 2 siblings in the same family are often affected but their parents are asymptomatic. Recently, chronic relapsing thrombotic thrombocytopenic purpura (CR-TTP), reported almost exclusively in adults, was shown to be caused by inherited or acquired deficiency in the activity of a plasma von willebrand factor-cleaving protease (vWF-CPase). The pathogenesis of USS is unknown, and a relationship between CR-YEP and USS has not been reported. We studied 3 unrelated USS patients (ST, SY, and KI) who presented with severe indirect neonatal hyperbilirubinemia. All 3 patients had undetectable vWF-CPase activity, and the inhibitors to vWF-CPase were all negative. In their parents with no clinical symptoms, vWF-CPase activities as a percentage of control samples (mother/father) were 17/20 for ST, 60/45 for SY, and 36/5.6 for KI. Thus, USS and vWF-CPase activity appear to be coinherited as autosomal recessive traits. Transfusion of fresh frozen plasma in 2 patients (ST and SY) resulted in the expected maximal increment of approximately 7% to 8% in vWF-CPase activity at 1 to 4 hours, but the levels became less than 3% within 2 days. After this decrease, platelet counts increased, plateaued in the normal range at 10 to 12 days, and declined thereafter. Thus, the 2 to 3 weeks of therapeutic benefit from plasma infusions will be discussed in relation to the intravascular lifetime of vWF-CPase.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
13/72. Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension.The management of idiopathic thrombocytopenic purpura (ITP) during pregnancy, especially with ongoing bleeding diathesis, has not been highlighted sufficiently in the literature. Aortocaval compression and reduction in uteroplacental circulation resulting in foetal hypoxia and acidosis, Mendelson's syndrome due to gravid uterus, trauma to airway with resultant haemorrhage and aspiration into lungs, compromised airway due to short neck, anasarca and heavy breast, limitation in using invasive monitoring and regional anaesthesia and uncontrolled bleeding leading to placental hypoperfusion and foetal hypoxia are some of the important risks. In the present case report, anaesthetic management for splenectomy during pregnancy complicated with pregnancy induced hypertension and bleeding diathesis secondary to ITP is described with reference to above risks.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
14/72. Papular-purpuric gloves-and-socks syndrome with bloody bullae.Papular-purpuric gloves-and-socks syndrome (PPGSS) is a disease characterized by itchy, painful acral erythema with edema, confluent papules, and purpura in a gloves-and-socks distribution and is associated with fever and mucosal lesions. parvovirus B19 and other viral infections have been proven to be causative agents of this syndrome. Its histological findings have been the non-specific ones of interface dermatitis. Here, we report a case of PPGSS in a 44-year-old man that we believe to be the first such case in japan. He developed, within one day, a painful edematous eruption with confluent papules and purpura on his hands and feet accompanied by high fever. A unique clinical manifestation in this case was multiple bloody bullae on the toes, which have not been previously described. Serological tests were negative for parvovirus B19, cytomegalovirus, and measles virus.- - - - - - - - - - ranking = 2keywords = purpura (Clic here for more details about this article) |
15/72. Sicklemia with multi-organ failure syndrome and thrombotic thrombocytopenic purpura.Acute multi-organ failure syndrome is a rare and life-threatening complication of patients with sickle cell disease. The syndrome appears to be reversed with prompt, aggressive exchange transfusion therapy. It has been attributed to widespread vascular occlusion due to micro-vascular red cell sickling. We present a case of severe multi-organ failure in a patient with sickle thalassemia and mild clinical course, who had clinical and laboratory features consistent with thrombotic thrombocytopenic purpura (TTP). The dramatic response to therapy with plasma exchange, a treatment often effective in TTP, suggests a similarity in pathophysiology of micro-vascular occlusion and multi-organ failure in sickle cell disease.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
16/72. Sebastian syndrome: report of the first case in a South American family.The Sebastian syndrome (SS) is a MYH9-related disorders, which are an extremely infrequent group of four autosomal dominant illnesses. SS consist of giant platelets, leukocyte inclusions and thrombocytopenia. To our knowledge, there are no case reports of this syndrome in south america. The propositus was a 35-year-old Argentine woman with a history of purpuric lesions in her lower limbs and thrombocytopenia. Idiopathic thrombocytopenia purpura (ITP) was previously diagnosed, but she did not respond to treatment with steroids. family history failed to provide any evidence of hearing loss, easy bruising, nephritis, renal failure or cataracts. The patient and 11 members of her family were evaluated. The diagnosis of SS was established by demonstrating giant platelets, thrombocytopenia and leukocyte inclusions in peripheral smear in two relatives and by peripheral smear and electronic microscopy in the propositus. MYH9-related disorders should be suspected whenever a patient has a low platelet count or a bleeding diathesis of unknown origin. In these cases, the history, carefully peripheral smear exam, immunocytochemistry and electronic microscopy will be of great help. Differentiation ITP with SS is important to avoid unnecessary diagnostic studies and treatments.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
17/72. Thrombotic thrombocytopenic purpura-like syndrome associated with systemic lupus erythematosus--combined treatment with plasmapheresis and fresh frozen plasma infusion.We report on a patient with systemic lupus erythematosus, who, during the course of the illness, developed thrombotic thrombocytopenic purpura. In this case, the coexistence of these two conditions was confirmed by laboratory and pathologic findings. The infusion of fresh frozen plasma with plasmapheresis reversed the course of thrombotic thrombocytopenic purpura.- - - - - - - - - - ranking = 6keywords = purpura (Clic here for more details about this article) |
18/72. Autoerythrocyte sensitization syndrome (psychogenic purpura).A case of autoerythrocyte sensitization syndrome is reported. This syndrome most often appears in young women who have an underlying emotional disorder; features include bizarre, tender ecchymotic lesions, which are most commonly located on the arms and legs. Systemic symptoms often accompany the onset of these lesions. A diagnosis of autoerythrocyte sensitization syndrome may be made in a patient who has the typical history and clinical picture of the syndrome and in whom a skin test with use of the patient's blood reveals a positive reaction.- - - - - - - - - - ranking = 4keywords = purpura (Clic here for more details about this article) |
19/72. Petechial glove and sock syndrome caused by parvovirus B19.The petechial glove and sock syndrome is a recently described febrile dermatosis characterized by acral pruritus, edema, pain, petechiae, and an enanthem of petechiae and erosions; these features suggest a viral origin. We report a typical case in a 36-year-old woman. IgM antibodies to human parvovirus B19 (PVB19) were present, and acute and convalescent IgG antibodies demonstrated seroconversion, which suggested recent infection with PVB19. Results of tests for other viral and bacterial agents were negative. These results strongly implicate PVB19 as an etiologic agent in the petechial glove and sock syndrome.- - - - - - - - - - ranking = 21.853725651593keywords = petechiae (Clic here for more details about this article) |
20/72. oral manifestations of papular-purpuric 'gloves and socks' syndrome due to parvovirus B19 infection: the first case presented in greece and review of the literature.Papular-purpuric 'gloves and socks' syndrome (PPGSS) is a novel, rare, self-limited dermatosis initially described in 1990. It is characterized by painful, pruritic edema and erythema, rapidly evolving to papular-purpuric lesions on the distal extremities, in a gloves-and-socks distribution, accompanied by fever and oral lesions such as petechiae, vesiculopustules and small erosions. parvovirus B19 has been implicated in most cases as the etiological factor. Herein we present the first case of PPGSS in a 42-year-old Greek man with von Willebrand disease. On admission the patient was febrile, and presented acral edema and erythema rapidly followed by purpuric lesions on the same sites, and palatal petechiae. Complete remission of the exanthem occurred 7 days after hospitalization. Clinical and laboratory evaluation including serologic tests and PCR, confirmed the presence of parvovirus B19. review of the existing literature on this novel syndrome and its association with parvovirus B19 is also presented.- - - - - - - - - - ranking = 21.853725651593keywords = petechiae (Clic here for more details about this article) |
| <- Previous || Next -> |