Cerebral vasculitis in a child with Henoch–Schönlein purpura and familial Mediterranean fever

In this case report, a 10-year-old girl with Henoch–Schönlein purpura (HSP) with severe central nervous system involvement and also having familial Mediterranean fever (FMF) is presented.     

Leptin levels in Henoch–Schönlein purpura

The objective of this paper is to assess the possible role of nitric oxide (NO) and leptin in Henoch–Schönlein purpura (HSP). We investigated the serum leptin and total nitrite levels in 22 children with HSP in the acute phase and after remission and in 20 age- and sex-matched healthy control. Serum leptin levels (nanograms per milliliter; median, min–max) were statistically higher in the acute phase (12.9, 9.1–19.5) than those in the remission phase (6.1, 3.7–10.5, p<0.001) and in the control group (4.9, 3.8–7.5, p<0.001). Also, serum nitrite levels (micromole per liter; median, min–max) were higher in children in the acute phase (45.0, 32.0–60.0) compared to those in remission phase (30.5, 23.0–48.0) and in the control group (29.5, 18.0–38.0) (p<0.001, p<0.001, respectively). There was a positive correlation between serum leptin and total nitrite levels (r=0.65, p<0.001). We have demonstrated that serum leptin and NO levels were increased during the acute phase in children with HSP, and returned to normal levels in remission. We suggest that leptin and NO may have a role in the immunoinflammatory process of HSP, especially in the acute phase. Further studies are needed to clearly establish the roles of leptin and NO in the pathogenesis of HSP.     

Scrotal involvement in an adult with Henoch–Schönlein purpura

Henoch–Schönlein purpura is a systemic vasculitis of unknown etiology usually affecting the pediatric age group and characterized by the clinical triad of non-thrombocytopenic palpable purpura, abdominal pain, and arthritis. There also may be varying degrees of renal involvement. The findings of scrotal involvement are not as well recognized. We describe a case of acute scrotal swelling as part of a 37-year-old male’s presentation of Henoch–Schönlein purpura, a presentation that has not been reported in this age group.

     

Adult T-cell lymphoma mimicking Henoch–Schönlein purpura

We report a male patient with adult T-cell lymphoma, who was initially diagnosed clinically as having Henoch–Schönlein purpura (HSP) with abdominal pain and specific purpura. Adult T-cell lymphoma-like cells were minimal and abdominal lymph nodes were transiently swollen, and the symptoms were improved by supportive management. Although the clinical course was compatible with HSP, the histological examination revealed infiltration of lymphocytes rather than neutrophils. Later he developed lymphoma and was treated with chemotherapy. This rare case suggests the importance of skin biopsies to seek the underlying pathology in adult HSP.     

Paroxysmal drastic abdominal pain with tardive cutaneous lesions presenting in Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. The diagnosis of HSP is difficult, especially when abdominal symptoms precede cutaneous lesions. We report a rare case of paroxysmal drastic abdominal pain with gastrointestinal bleeding presented in HSP. The diagnosis was verified by renal damage and the occurrence of purpura.     

Antioxidant enzyme activities,lipid peroxidation,and total antioxidant status in children with Henoch–Schönlein purpura

The aim of this study was to assess the role of oxidative stress in the pathogenesis of Henoch–Schönlein purpura (HSP) vasculitis. The activities of catalase (CAT), arylesterase (ARYL), and paraoxonase (PON) as antioxidant enzymes and serum malondialdehyde (MDA) level as an indicator of lipid peroxidation, together with total antioxidant status (TAS), were measured in 29 children with HSP (mean age 9.3?±?2.7 years), both at the onset of the disease and at the remission period and in matched controls. Active-stage HSP had significantly higher MDA level (15.5?±?7.3 vs 7.8?±?3.9 nmol/l, respectively, P?P?P?=?0.042), ARYL (158?±?39 vs 212?±?52 U/l, P?P?=?0.002) activities compared with the control subjects. Although CAT (P?>?0.05) and PON (P?>?0.05) activities were found to be similar between active and remission stages of HSP, the active stage of the disease had significantly lower ARYL (P?=?0.011) and TAS (P?=?0.006) and higher MDA (P?r?=?0.433, P?=?0.019) and between CAT and C-reactive protein (r?=?0.386, P?=?0.035) in the active stage of HSP. No significant differences were detected in oxidant/antioxidant parameters between patients with or without renal, gastrointestinal, or joint involvement (P?>?0.05). Increased oxidative stress and lipid peroxidation may play important roles in the pathogenesis of HSP vasculitis. Antioxidant therapeutic interventions in long-lasting vasculitis and risk of atherosclerosis secondary to increased oxidant stress remain to be investigated.     

Henoch–Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome

We report a young female patient with Henoch–Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae.     

Henoch–Schönlein purpura: Hitting the ‘target’ on CT

This image highlights the common findings on CT in Henoch-Schonelin Purpura, namely 'target sign' and comb sign. We discuss the common CT findings in this disease.     

Severe gastrointestinal involvement in adult-onset Henoch–Schönlein purpura associated with clarithromycin-resistant Helicobacter pylori infection

Background

Henoch–Schönlein purpura (HSP) is an uncommon vasculitis in adults. Gastrointestinal involvement is part of the classical tetrad and can present as bleeding. Helicobacter pylori infection in the setting of HSP has been reported a few times in the literature and may be involved in the pathogenesis of this disease as a triggering agent.

Successful Treatment of mesenteric vasculitis caused by henoch–Schönlein purpura with methylprednisolone pulse therapy

Although mesenteric vasculitis due to Henoch–Schönlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.     

MEFV gene mutations in children with Henoch–Schönlein purpura and their correlations—do mutations matter?

Clinical Rheumatology - To explore the frequency of MEFV gene mutations in children with Henoch–Schönlein purpura who had no prior familial Mediterranean fever diagnosis and to evaluate...     

Henoch-Schönlein purpura presenting as terminal ileitis and complicated by thrombotic microangiopathy

We report here on a 40-year-old woman with abdominal pain, low-grade fever, and diarrhea in whom the cutaneous features of Henoch–Schönlein purpura (HSP) appeared only a few days after acute abdominal symptoms. Endoscopy showed terminal ileitis, and histopathological examination of a biopsy of the ileum showed a picture of IgA vasculitis. The clinical course was further complicated by the development of microangiopathic hemolytic anemia, thrombocytopenia, and severe renal failure.     

Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch–Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch–Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch–Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.     

The clinical spectrum of Henoch–Schönlein purpura in children: a single-center study

Clinical Rheumatology - Henoch–Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients...     

Diagnostic value of nailfold capillaroscopy changes in Henoch–Schönlein purpura children with gastrointestinal or renal involvement

Aim of the work: Capillaroscopy is known as a non-invasive, safe, and valuable diagnostic tool to assess the small vessels of the microcirculation in the nailfold. This study aimed to determine the diagnostic value of capillaroscopy in children with Henoch-Schönlein purpura (HSP) and major organ (gastrointestinal or renal) involvement which could increase the risk of complications. Patients and method: This study was conducted on 32 HSP children referred to the rheumatology ward of Children's Medical Center in Tehran, Iran, from 2019 to 2020, as well as 30 healthy controls. The vascular changes of the nailfold were assessed through capillaroscopy. Results: The mean age of the patients was 6.4 ± 2.2 years (3–11 years), and the female/male ratio was 1:1.7 (12 females and 20 males). The main finding of nail-fold capillaroscopy was abnormal morphology in 56.2 % of patients (vs. 6.7% control). A significant difference was seen between patients and control in terms of capillary morphology and architecture (p < 0.05). Abnormal capillary density and diameter tended to be more common in patients than in control (p > 0.05). Seven patients had gastrointestinal or renal involvement (21.9%). A high negative predictive value and low positive predictive value of density, architecture, and diameter of capillaries, were noted between patients with and without gastrointestinal and renal involvement. Conclusion: The role of microvascular involvement in children with HSP is emphasized. Changes in these vessels could be an image of this small vessel vasculitis to reflect major organ invovlement and thus avoid future complications of the disease.