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We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura. She went on to develop hemoptysis and progression of the rash into erosive bullae. Investigations revealed that she was ANCA positive and had pauci-immune glomerulonephritis. Given her upper airway, pulmonary and renal involvement, and antineutrophil cytoplasmic antibodies positivity, a definitive diagnosis of a severe form of GPA was made. GPA is a chronic relapsing, life threatening vasculitis that predominantly affects small vessels. Conclusion: Our case demonstrates that GPA can present initially with nonspecific symptoms, including extensive dermatological involvement, leading to diagnostic confusion, and delays in treatment. In the case of a severe peripheral rash in the juvenile population and/or resistant upper airway symptoms, it is vital to consider a diagnosis of GPA to avoid serious organ or life threatening consequences. [ABSTRACT FROM AUTHOR]

An 11-day-old neonate presented with purpura fulminans and was subsequently diagnosed with galactosemia. Neonatal purpura fulminans occurs predominantly in patients suffering from inherited protein C deficiency or disseminated intravascular coagulation associated with septicemia. Hemostatic changes in patients with liver disease may result in bleeding or, rarely, thrombosis. We suppose that in the present patient, deficiency of protein C, secondary to liver disease, was responsible for the development of purpura fulminans. Treatment consisted of blood and blood products and galactose-free formula. The patient recovered with residual mild psychomotor retardation and the lesions with minimal scarring. In conclusion, galactosemia also should be kept in mind as an uncommon cause of purpura fulminans in newborn infants. [ABSTRACT FROM AUTHOR]

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. In this review, the main clinical features and complications are described. Although most features are self-limiting, renal disease is the most likely to result in long-term morbidity. Treatment of HSP nephritis is controversial, and the evidence for both prevention and treatment of established disease is reviewed. Follow-up for children presenting with HSP should be for at least 6 months and should include regular urine testing for proteinuria and haematuria and a blood pressure measurement. Women with a history of HSP during childhood are at increased risk of complications (such as proteinuria and hypertension) during pregnancy and should be monitored closely. This paper describes current practice with regard to investigation and management and proposes a practical care pathway of the management of a child with HSP. [ABSTRACT FROM AUTHOR]

A 13-month-old Japanese female with Haemophilus influenzae type b meningitis presented with unusually severe septic shock and cerebral infarction in half a day of fever. The initial therapy of plasma-derived activated protein C (Anact C) led to an impressive effect on the aggressive condition. However, purpura fulminans and the consistent decline of plasma protein C activity (<20%) required prolonged activated protein C therapy and gene analysis. The patient carried a novel heterozygous mutation of PROC (exon 4; 335 GAC>TAC, Asp46Tyr). This is the first report of infectious purpura fulminans in a protein C-deficient heterozygote. The clinical onset and treatment course adequately corroborated the aggravated immune/hemostatic reactions and the cytoprotective effects of activated protein C replacement in human heterozygous protein C deficiency. The monitoring of plasma protein C activity and sufficient administration of activated protein C product could improve the outcome of severe sepsis in children. [ABSTRACT FROM AUTHOR]

There is increasing recognition that Henoch-Schonlein purpura may present in an atypical form in which gastrointestinal symptoms may predominate, and classic cutaneous changes may be delayed or absent. This may lead to significant diagnostic delay. We report the case of a 9-year-old girl who presented acutely with life-threatening gastrointestinal haemorrhage from multiple intestinal sites, with no skin rash and only mild evidence of renal involvement. Henoch-Schonlein purpura was confirmed by finding IgA deposition on vessels within gastric and duodenal mucosa, while immunohistochemistry also identified dense focal T cell infiltration in gastric mucosa and within duodenal epithelium. After initial stabilisation, the patient became shocked due to further gastrointestinal haemorrhage. Isotope bleeding scan identified multiple bleeding sites. Her endoscopically confirmed gastritis was sufficiently severe to preclude corticosteroids, and she was thus treated with intravenous immunoglobulin. This therapy induced prompt and sustained resolution of symptoms, and she has remained well since. Our patient's response concords with previous reports in corticosteroid-resistant cases to suggest that severe intestinal Henoch-Schonlein purpura may respond preferentially to intravenous immunoglobulin (IVIG) therapy. In severe cases where there is significant gastritis, IVIG provides an effective alternative to corticosteroids that may be employed as first-line therapy. [ABSTRACT FROM AUTHOR]

We report on identical twin sisters with systemic lupus erythematosus and lupus nephritis after initial presentation as idiopathic thrombocytopenic purpura. The patients had diverse clinical signs, symptoms and pathological findings of lupus nephritis. The changes in antinuclear antibodies and anti-double-stranded DNA antibodies were quite different. We conclude that even with an identical genetic background and the same environment, the expression of systemic lupus erythematosus and subsequent progression to lupus nephritis in twins was distinct, and antinuclear antibodies and anti-double-stranded DNA antibodies are helpful for clinical monitoring. [ABSTRACT FROM AUTHOR]

Unlabelled: Risk factors of renal involvement and significant proteinuria in patients with Henoch-Schönlein purpura (HSP) were retrospectively evaluated by univariate and multivariate analyses. The analysis was performed in 134 patients with HSP. Renal involvement was found in 65 patients (49%) and 97% of the renal involvement was found within 3 months of disease onset. Moderate or severe proteinuria was recognised in 25 patients. A univariate analysis revealed that an age of more than 4 years at the onset, severe abdominal pain with gastrointestinal bleeding, persistent purpura over a month, coagulation factor XIII activity < 80%, and treatment with factor XIII concentrate were associated with developing renal involvement. A multivariate analysis showed that severe abdominal symptoms, an age of more than 4 years, and persistent purpura increased the risk of renal involvement. Risk factors of moderate or severe proteinuria were also examined. The risk factors in a univariate analysis were severe abdominal symptoms, persistent purpura, decreased factor XIII activity, treatment with steroids, and treatment with factor XIII concentrate. Of those, persistent purpura, treatment with factor XIII concentrate, and factor XIII activity < 80% were associated with significant proteinuria in a multivariate analysis. Among the patients with severe abdominal symptoms, factor XIII activity was significantly decreased in patients with significant proteinuria compared to other patients without significant proteinuria.Conclusion: Long-term prognosis of Henoch-Schönlein purpura is dependent on the severity of renal involvement. In those patients who have the risk factors of renal involvement, especially significant proteinuria, close attention should be paid to a urinalysis for at least 3 months from the onset of the disease. [ABSTRACT FROM AUTHOR]

Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP. [ABSTRACT FROM AUTHOR]

Presents information on a study which examined the manifestation of immunoglobulin A nephropathy in several patients with end-stage renal failure. Age distribution of the manifestation; Clinical features; Relation with Henoch-Schoenlein purpura.

Unlabelled: More than 10% of children surviving septic shock with purpura have skin necrosis or limb ischaemia (SNLI.). Among 44 children consecutively admitted to our pediatric intensive care unit, 35 (80%) survived, 6 of them (17%) developed SNLI (defined as the need of a surgical procedure). Two timed haemostasis measurements included the determination of coagulation factors, protein C (PC), protein S (PS), C4b binding protein (C4bBP), antithrombin (AT), and plasminogen activator inhibitor 1 (PAI-1). Two severity scores and CRP levels were determined at admission. Children with SNLI and without SNLI were compared. On admission, severity scores, and AT, PC, PS, C4bBP levels were similar in both groups with and without SNLI. Prothrombin time (23% vs 34%; P < 0.01), factor VII+X (20% vs 31%; P = 0.05) and factor VII (0% vs 19%; P < 0.01) were lower in the group with SNLI. The 2nd sample showed no difference between the two groups. Kinetics of haemostatic abnormalities were no different between the two groups.Conclusion: In this series, the only difference between the two groups was lower factor VII levels in children with skin necrosis or limb ischaemia. This suggests the benefit of tissue factor pathway inhibitor administration as an adjunctive therapy to prevent skin necrosis or limb ischaemia. Further studies including more children are needed to determine the potential effects of treatments such as protein C, antithrombin, and plasminogen activator inhibitor antibody administration, and to advocate tissue factor pathway inhibitor in preventing skin necrosis or limb ischaemia. [ABSTRACT FROM AUTHOR]

Chronic relapsing thrombotic thrombocytopenic purpura (CRTTP) is the rarest type of TTP, usually presenting in childhood. The aetiology is still not fully explained. The disorder is associated with the presence in plasma of unusually large von Willebrand factor (UlvWF) multimers that are especially prominent between episodes. CRTTP can be prevented by periodic plasma transfusions. We report three children with congenital CRTTP who have been successfully treated and maintained in prolonged remission by the prophylactic use of fresh frozen plasma without concurrent plasmapheresis. Two of the patients are sibs. [ABSTRACT FROM AUTHOR]

Unlabelled: Two unrelated female infants with homozygous protein C (Pr C) deficiency are reported. Both are of U.K. Pakistani origin and in each case the parents are consanguinous. A previous sibling had died in each family. Both sets of parents were shown to be carriers. The concentration of Pr C in both infants was low at birth. Both developed necrotic skin lesions (purpura fulminans) and responded well to Pr C concentrate. Both are developing normally although one has visual impairment due to retinal artery thrombosis which occurred before treatment was commenced. Both infants are treated with intravenous Pr C concentrate administered daily by the parents at home. Studies of the half-life of exogenous Pr C in one of the patients has shown an increase from 2.7 to 10.8 h during the course of treatment thus enabling it to be administered once daily while still maintaining effective plasma concentrations. In the other patient half-life has fluctuated but Pr C is also given once daily. This is the first report of this condition being treated in this way in the United Kingdom.Conclusion: Infusion of Pr C is a safe and efficient way of treating infants with homozygous Pr C deficiency in the medium term. [ABSTRACT FROM AUTHOR]

Henoch-Schönlein purpura is the most common systemic vasculitis during childhood. Many antigenic stimuli and infectious agents have been proposed as a trigger of the disease. Group A beta-hemolytic streptococcus is the most common proposed infectious agent as a trigger for Henoch-Schönlein purpura. We report a 9-year-old boy who has Henoch-Schönlein purpura and acute rheumatic fever with complete atrioventricular block. [ABSTRACT FROM AUTHOR]

Describes a case of severe aplastic anaemia complicating Sjögren syndrome in a child. Medical background of the patient; Physical examination of the condition of the patient; Treatments administered to the patient.

Reversible posterior leukoencephalopathy syndrome (RPLS) is a recently described disorder with typical radiological findings of bilateral grey and white matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with RPLS are adults and it is rare in children. In this report, two patients with RPLS are presented. In the first patient the primary diagnosis was acute post-streptococcal glomerulonephritis, a known cause of RPLS both in adults and in children. The second patient had Henoch Schönlein purpura.Conclusion:These patients are presented to highlight the importance of reversible posterior leukoencephalopathy syndrome. As the spectrum of associated diseases is diverse, paediatricians must be aware of this syndrome in order to initiate appropriate management. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article on Henoch-Schonlein purpura is presented.

Henoch-Schonlein purpura (HSP) is an inflammatory vasculitis involving the skin, joints, gastrointestinal (GI) tract, and kidneys. This is the first case report describing a 5-year-old girl with HSP presenting duodenal involvement which might be associated with superior mesenteric artery syndrome (SMAS). [ABSTRACT FROM AUTHOR]

Discusses neonatal purpura due to Neisseria meningitidis serogroup C infection. Presentation of a neonate with meningococcaemia and a petechian and purpuric rash; Mother's urinary tract infection during the end of pregnancy; Uncertainty regarding the exact source of the infection.

Compares the results of three studies about fibronectin in meningococcal disease. Levels of fibronectin in children suffering from septic shock; Relation of fibronectin levels and the severity of purpura; Relation of coagulation inhibitor levels with skin lesions.

The article presents a letter to the editor in response to the article "A Case of Henoch-Schonlein Purpura and Rheumatic Carditis with Complete Atrioventricular Block," published in the 2006 issue of the "European Journal of Pediatrics."

Abstract Homozygous congenital protein C deficiency is accompanied by severe thrombophilia. Thrombotic events can be reduced in number and severity by lifelong oral anticoagulation therapy. A 4-year-old boy was first diagnosed as having severe congenital homozygous protein C deficiency during the neonatal period when purpura fulminans occurred as the first manifestation of thrombosis. From this time he had been treated with phenprocoumon (INR 3.5-4.5). During an infection of the upper respiratory tract he developed signs of a new episode of purpura fulminans (INR 2.6). Additional anticoagulation therapy with LMW heparin (Clexane(R)) and protein C concentrate was given while the oral anticoagulation therapy was continued. On the third day of this episode the boy suffered from pain of unknown origin. MRI of the abdomen and of the pelvis revealed nontraumatic osteonecrosis of the hip joint. After a few weeks of immobilisation no special treatment was necessary. One year later he was able to walk with no problem. Conclusion Aseptic osteonecrosis of the hip joint is associated with a variety of disorders including vascular thrombosis and haemorrhage. Especially young children and handicapped patients with thrombophilia and pain of unknown origin are suspected to have thrombosis in atypical regions. [ABSTRACT FROM AUTHOR]

Describes the case of a patient with purpura fulminans complicating pneumococcal sepsis. Symptoms associated with purpura fulminans; Drugs administered on the patient.