Immunoglobulin A nephropathy in horseshoe kidney: case reports and literature review.
Hu Panpan,Jin Meiling,Xie Yuansheng,Chen Pu,Zhang Xueguang,Yin Zhong,Cai Guangyan,Chen Xiangmei
Nephrology (Carlton, Vic.)
Horseshoe kidney is the most common congenital renal fusion anomaly. Immunoglobulin A nephropathy is a common glomerulonephritis worldwide. However, the co-occurrence of these diseases had not been reported in the literature. We report the first two cases with the occurrence of immunoglobulin A nephropathy in horseshoe kidney. The first case was a 26-year-old male with hypertension and proteinuria (1.4 g/24 h), his pathological finding was primary immunoglobulin A nephropathy. The second case was a 15-year-old female who presented with recurrent peliosis on bilateral lower extremities, haematuria and proteinuria (1.7 g/24 h). Her renal biopsy finding was Henoch-Schonlein purpura nephritis (secondary immunoglobulin A nephropathy). In both cases, renal biopsy was performed by experienced doctors under ultrasonic guidance at the renal upper pole and no postoperative complications were observed. After they were treated based on the renal pathological findings for 6 months, urine protein excretion decreased significantly and blood pressure and serum creatinine stabilized. It is possible that immunoglobulin A nephropathy occurs in a horseshoe kidney patient. Renal biopsy may be valuable and viable for horseshoe kidney patients with heavy proteinuria to identify pathologic type of glomerulopathy and to guide treatment, if renal biopsy is performed by experienced doctors at the renal upper pole under renal ultrasonic guidance.
HENOCH-SCHÖNLEIN PURPURA NEPHRITIS FOLLOWING INFLUENZA VACCINATION: A CASE REPORT AND REVIEW OF THE LITERATURE
Liu Jing-Yi,Wu Qiang-song,Liu Min,Wang Lan,Gao Yan-hong,Li Shen-sheng
The Southeast Asian journal of tropical medicine and public health
We reported a 16-year-old boy who developed Henoch-Schönleinpurpura (HSP) 15 days after receiving a seasonal influenza vaccine. His symptomsimproved temporally with treatment but he developed HSP nephritis(HSPN) that relapsed multiple times over the following three years. This caseof Henoch-Schönlein purpura may have been due to the seasonal influenzavaccine. The mechanism for this association is unclear. Practitioners shouldbe aware of this possible complication.
Perpetually perplexing pediatric patients: it's complicated: part 2.
Block Stan L
Many pediatric patients that you encounter will challenge your diagnostic and therapeutic skills. As the following cases will illustrate, some will perplex you with their esoteric or convoluted illnesses despite your best attempts. Although their initial presentation may often be mundane, thorough history taking and physical examination, and a keen "clinical intuition" will be invaluable.
Notes from the Field: Outbreak of Hand, Foot, and Mouth Disease Caused by Coxsackievirus A6 Among Basic Military Trainees - Texas, 2015.
Banta Jonathan,Lenz Brittany,Pawlak Mary,Laskoski Kelly,Seykora Caitlin,Webber Bryant,Yun Heather,Ritchie Simon
MMWR. Morbidity and mortality weekly report
On July 7, 2015, a man aged 22 years reported to sick call during basic military training at Lackland Air Force Base (AFB), Texas. He had erythematous, crusted papulovesicular lesions on the extensor surfaces of the upper and lower extremities. The patient was afebrile and otherwise well, and was evaluated later that day by the dermatology service. A viral infection was considered most likely because of the patient's age, absence of fever or constitutional symptoms, and the distribution and morphology of the lesions. The initial differential diagnosis included Henoch-Schönlein purpura, parvovirus B19, and Rocky Mountain spotted fever. However, the clinical signs, including the unique morphology and distribution of grouped vesicles and papules was suggestive of hand, foot, and mouth disease (HFMD), although the patient did not have oral lesions and reported no contact with another person with HFMD. A viral culture and punch biopsy of one of the lesions were obtained.
A case of acute encephalophathy with residual neurological sequelae induced by immunoglobulin A vasculitis.
Hamano Hiroki,Matsushige Takeshi,Inoue Hirofumi,Hoshide Madoka,Kobayashi Hikaru,Kohno Fumitaka,Oka Momoko,Ichiyama Takashi,Ohga Shouichi,Ouchi Kazunobu,Hasegawa Shunji
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
Immunoglobulin A vasculitis (IgAV) occasionally induces central nervous system (CNS) involvement, which is usually transient with no sequelae except for hemorrhagic stroke. It is thought to be useful to measure serum and cerebrospinal fluid (CSF) cytokine levels for better understanding the pathological condition in encephalopathy, but there have been no reports in acute encephalopathy with IgAV. We describe an 8-year-old boy with IgAV who had neurological sequelae after complication of acute encephalopathy, focusing on the cytokine profiles and unique biphasic findings of magnetic resonance imaging. He presented with status epilepticus and mildly intensified area in the occipital lobe on the fluid-attenuated inversion recovery view. Arterial spin labeling (ASL) revealed the reduction of cerebral blood flow in the left hemisphere. On day 5 of illness, these abnormal findings disappeared, but delayed hyperintensity lesions on diffusion-weighted images newly emerged. Furthermore, CSF interleukin (IL)-6 levels markedly increased without elevated levels of IL-10 during the acute phase of disease. He suffered from long-lasting hemiparesis and intellectual impairment. In conclusion, acute encephalopathy with IgAV could cause neurological sequelae by prolonged seizure, and elevated IL-6 in CSF and laterality of cerebral blood flow in ASL might be useful to predict the prognosis of CNS dysfunction of IgAV.
What is that rash?
Speirs Lynne,McVea Steven,Little Rebecca,Bourke Thomas
Archives of disease in childhood. Education and practice edition
CASE HISTORY:A healthy 15-month-old girl presented to the emergency department with a 24-hour history of fever and rash. The initial blanching rash developed into non-blanching areas with associated leg swelling. She had received no recent medications, had no known drug allergies and no unwell contacts.On examination, she was feverish at 38.6°C, capillary refill time was <2 s with warm peripheries, heart rate 169 bpm and blood pressure 94/59 mm Hg. A palpable purpuric rash was evident on all four limbs and face (figure 1) although the trunk was spared. Her legs were tense and oedematous to the knee.edpract;103/1/25/EDPRACT2016311782F1F1EDPRACT2016311782F1Figure 1Rash at presentation.Initial investigations: Haemoglobin level: 131 g/L, white cell count: 16.6×10/L, neutrophils: 11.1×10/L and platelets: 407×10/LCoagulation screen: normalC reactive protein level: 20 mg/LLactate level: 1.7 mmol/LIntravenous ceftriaxone was commenced following blood culture and meningococcal PCR. The following day, while remaining systemically well, she developed a vesicular rash on her trunk and back (figure 2).edpract;103/1/25/EDPRACT2016311782F2F2EDPRACT2016311782F2Figure 2Vesicular rash. QUESTIONS:What is the diagnosis? Henoch-Schonlein purpura (HSP)Meningococcal septicaemiaAcute haemorrhagic oedema of infancy (AHOI)Vasculitic urticariaGianotti-Crosti syndromeWhat further investigation is required? Check viral serology including Epstein-Barr virus and hepatitis B virusComplement levels and autoimmune screenSkin biopsyLumbar puncture and audiologyNo further investigationHow should this child be managed? Complete 7 days of ceftriaxone treatmentOral aciclovirOral steroidsRegular follow-up with urinalysis and blood pressure monitoringStop antibiotics if cultures were negative at 48 hours and discharge▪▪.
Petechiae and purpura: the ominous and the not-so-obvious?
Block Stan L
Petechiae and purpura are among the most alarming findings a pediatrician will commonly observe in the office. Severity of illness can range from a temper tantrum, to common viral infections, to the most deadly infections and diseases. To avoid many of the pitfalls in diagnosis, practitioners will need to be thorough in history taking, assessing fever and immunization status, and physical examination. In addition, a few simple laboratory tests will usually be needed and possibly a manual differential.
A 19-year old man with IgA vasculitis after vaccination.
Malek Alexandre,Gomez-Villegas Sara Isabel,de la Hoz Alejandro,Nowbakht Cima,Arias Cesar A
The Brazilian journal of infectious diseases : an official publication of the Brazilian Society of Infectious Diseases
A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.
Immunoglobulin A Vasculitis Complicated with Clostridium difficile Infection: a Rare Case Report and Brief Review of the Literature.
Cojocariu Camelia,Stanciu Carol,Ancuta Codrina,Danciu Mihai,Chiriac Stefan,Trifan Anca
Journal of gastrointestinal and liver diseases : JGLD
Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary. We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature.
Hemorrhagic Bullous Henoch-Schönlein Purpura: Case Report and Review of the Literature.
Nothhaft Matthias,Klepper Joerg,Kneitz Hermann,Meyer Thomas,Hamm Henning,Morbach Henner
Frontiers in pediatrics
Henoch-Schönlein Purpura (HSP) or IgA vasculitis is the most common systemic vasculitis of childhood and may affect skin, joints, gastrointestinal tract, and kidneys. Skin manifestations of HSP are characteristic and include a non-thrombocytopenic palpable purpura of the lower extremities and buttocks. Rarely, HSP may initially present as or evolve into hemorrhagic vesicles and bullae. We present an otherwise healthy 5-year-old boy with an acute papulovesicular rash of both legs and intermittent abdominal pain. After a few days the skin lesions rapidly evolved into palpable purpura and hemorrhagic bullous lesions of variable size and severe hemorrhagic HSP was suspected. A histological examination of a skin biopsy showed signs of a small vessel leukocytoclastic vasculitis limited to the upper dermis and direct immunofluorescence analysis revealed IgA deposits in vessel walls, compatible with HSP. To further characterize the clinical picture and treatment options of bullous HSP we performed an extensive literature research and identified 41 additional pediatric patients with bullous HSP. Two thirds of the reported patients were treated with systemic corticosteroids, however, up to 25% of the reported patients developed skin sequelae such as hyperpigmentation and/or scarring. The early use of systemic corticosteroids has been discussed controversially and suggested in some case series to be beneficial by reducing the extent of lesions and minimizing sequelae of disease. Our patient was treated with systemic corticosteroids tapered over 5 weeks. Fading of inflammation resulted in healing of most erosions, however, a deep necrosis developing from a large blister at the dorsum of the right foot persisted so that autologous skin transplantation was performed. Re-examination 11 months after disease onset showed complete clinical remission with re-epithelialization but also scarring of some affected areas.
Mycoplasma pneumoniae as a trigger for Henoch-Schönlein purpura in children.
Kuźma-Mroczkowska Elżbieta,Pańczyk-Tomaszewska Małgorzata,Szmigielska Agnieszka,Szymanik-Grzelak Hanna,Roszkowska-Blaim Maria
Central-European journal of immunology
Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis. The majority of extrapulmonary symptoms are associated with skin changes such as exanthematous skin eruptions, erythema nodosum, urticaria, Stevens-Jonson syndrome. M. pneumoniae stimulates production of the interleukins and tumor necrosis factor (TNF) α and can cause vasculitis. Henoch-Schönlein purpura (HSP) is a leucoclastic vasculitis that affects small vessels. Clinical manifestations of HSP include typical rash, arthritis, gastrointestinal and sometimes renal involvement. The main feature in HSP is abnormal IgA deposits in vessel walls. Circulating abnormal glycosylated IgA 1 and IgG antibodies form immune complexes: IgA1-IgG and anti-IgA 1. Immune complexes activate cytokines, parts of complement and influence directly the endothelium. We report cases of three children with Henoch-Schönlein purpura with prolonged and recurrent skin and joint changes. The serological analysis (positive serum IgM) confirmed Mycoplasma pneumoniae infection. Treatment with clarithromycin caused complete regression of disease. We suggest that in the case of prolonged symptoms of vasculitis due to Henoch-Schönlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease.
Liver chemistry in new-onset Henoch-Schönlein syndrome.
Rosti Giulia,Milani Gregorio P,Laicini Emanuela A,Fossali Emilio F,Bianchetti Mario G
Italian journal of pediatrics
BACKGROUND:Henoch-Schönlein syndrome is a systemic small-vessel leukocytoclastic vasculitis that usually present with cutaneous, gastrointestinal, articular and renal manifestations. Little is known on liver involvement in this syndrome. This study investigated liver chemistry and creatine kinase in Henoch-Schönlein children. CASE PRESENTATION:Alanine aminotransferase, aspartate aminotransferase, γ-glutamyltransferase, lactate dehydrogenase, total bilirubin, prothrombin time and creatine kinase were assessed in 75 consecutive pediatric patients (41 boys and 34 girls aged from 2.9 to 17 years) with new-onset Henoch-Schönlein syndrome. Mildly altered values were found in 7 (9%) patients (5 boys and 2 girls aged from 3.3 to 17 years). In the mentioned cases, all tests returned to normal at a 2-4-week follow-up. CONCLUSIONS:This preliminary study points out that altered and self-remitting liver parameters occur in approximately 10% of children with Henoch-Schönlein syndrome.
Henoch-Schönlein purpura: hitting the 'target' on CT.
Dhir Varun,Pinto Benzeeta,Anakutti Hari Prasad,Yadav Mukesh Kumar,Sharma Shefali,Sharma Aman
Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology
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.
Penile Skin Involvement as the First Presentation of Henoch-Schonlein Purpura Report of Nine Cases and Review of Literature.
Paydary Koosha,Emamzadeh Fard Sahra,Mahboubi Amir Hassan,Ziaee Vahid,Moradinejad Mohammad Hassan,Kajbafzadeh Abdol-Mohammad
Iranian journal of pediatrics
INTRODUCTION:Involvement of penis is a rare presentation in henoch-schonlein purpura (HSP). The presentations are mainly due to the deposition of immunoglobulin A (IgA) into the vessel walls. In this report, we present the clinical history of nine HSP cases that presented with penile skin involvement. CASE PRESENTATION:All patients were referred in the acute phase of HSP. Penile skin involvement was evident as erythema, edema, ecchymosis, or induration of prepuce and/or penile shaft, that appeared simultaneously with skin rash in seven patients. Gastrointestinal involvement was positive in six patients. Patients were treated with steroids and follow up visits were normal except for one patient that developed crescentic glomerulonephritis. CONCLUSIONS:We present nine cases of HSP with penile involvement in order to indicate another rare aspect of HSP and its possible complications as well as its appropriate treatment.
Late Relapse of Henoch-Schönlein Purpura in an Adolescent Presenting as Severe Gastroduodenitis.
Rubino Chiara,Paci Monica,Resti Massimo,Lionetti Paolo,Trapani Sandra
Frontiers in pediatrics
Henoch-Schönlein purpura is a systemic vasculitis, commonly affecting children. Gastrointestinal manifestations are observed in 50-75% of patients; it is well known they may occur before skin lesions in about 20% of cases during the first vasculitic episode. Relapses occur in about one third of patients, typically within 4 months from the initial presentation and with milder symptoms. We report the case of a 17-year old girl with an atypical relapse of Henoch-Schönlein purpura, presenting with acute abdominal symptoms 5 years after the first episode. Esophagogastroduodenoscopy showed duodenal multiple hyperemic and hemorrhagic lesions. To our knowledge this is the first case of hemorrhagic-erosive duodenitis representing a relapse of Henoch-Schönlein purpura occurring several years after the initial episode. Duodenojejunal inflammation should be considered as primary manifestation of Henoch-Schönlein purpura, not only during the first episode, but also in relapses. Endoscopy can be helpful for differential diagnosis, especially in patients with atypical manifestations. Further studies are needed to evaluate risk factors for Henoch-Schönlein purpura recurrence and the possible role of fecal calprotectin as an early marker for gastrointestinal involvement.
Hemophagocytic lymphohistiocytosis secondary to Varicella zoster infection in a child with Henoch-Schönlein purpura.
Gur Gokce,Cakar Nilgun,Uncu Nermin,Ayar Ganime,Basaran Ozge,Taktak Aysel,Koksoy Adem Yasin,Acar Banu,Caycı Fatma Semsa
Pediatrics international : official journal of the Japan Pediatric Society
Hemophagocytic lymphohistiocytosis (HLH) is a fatal, hyper-inflammatory syndrome that is characterized by untimely activation of macrophages, and manifests as cytopenia, organ dysfunction, and coagulopathy. Secondary HLH can be associated with infection, drugs, malignancy, and transplantation, and is mostly triggered by infection. Herein, we report the case of a patient with Henoch-Schönlein purpura (HSP) who developed severe HLH secondary to Varicella zoster infection.
Henoch-Schönlein purpura with c-ANCA antibody in an adult.
Torraca Pedro de Freitas Silva,Castro Bruna Corrêa de,Hans Günter
Anais brasileiros de dermatologia
The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.
[Henoch-Schonlein purpura involving the penis: a case report].
Croche Santander Borja,Campos Elena,Sánchez Adela,Marcos Laura,Díaz Isabel,Toro Cristóbal
Archivos argentinos de pediatria
Schonlein-Henoch purpura accounts for the majority of cases of systemic vasculitis in children. Classical presentation is characterized by palpable purpura, glomerulonephritis, arthralgias and abdominal pain. Although genitourinary manifestations, in form of testicular and scrotal involvement, have been widely described, penile involvement remains an extremely rare complication. We report a case of a 6-year-old boy who presented with purpuric rash on the glans, prepuce and penile shaft, with painful edema in the penile region. He also had a 3-days history of fever, palpable purpuric rash on the buttocks and lower extremities along with right wrist pain. He was admitted with the diagnosis of Schonlein-Henoch purpura with penile involvement. After 2 days on oral steroids therapy (prednisone) a marked improvement was observed.
Management of intestinal bleeding with single-dose cyclophosphamide in Henoch-Schönlein purpura.
Uluca Ünal,Ece Aydın,Şen Velat,Yel Servet,Tan İlhan,Karabel Duran
Pediatrics international : official journal of the Japan Pediatric Society
In these case series, we report on six children (3 girls, 3 boys) aged 5-13 years with Henoch-Schönlein purpura (HSP) who developed severe gastrointestinal (GI) bleeding resistant to both 2 mg/kg or pulse (10-30 mg/kg) i.v. methylprednisolone. All patients responded to single-dose (500 mg/m(2) ) i.v. cyclophosphamide (CPA) and none of them developed new GI bleeding after CPA treatment. No patients required surgical intervention. Single high-dose CPA may be beneficial in HSP with severe GI involvement, in which bleeding is non-responsive to high-dose steroids.
Henoch-Schönlein purpura associated with Strongyloides stercoralis infection.
Janković Sveta,Nikolić Marijana,Simović Aleksandra,Vujić Ana
INTRODUCTION:Henoch-Schönlein purpura (HSP) is a small blood vessel vasculitis, which usually manifests during childhood. The exact cause of the disease is unknown. CASE REPORT:We reported a 14-year-old girl who had been admitted to our clinic due to the appearance of red macules on her extremities and face, vomiting, and pain in the abdomen and joints. The patient was initially diagnosed with Henoch-Schönlein purpura. At the end of the fourth week of illness, larvae of Strongyloides stercoralis were detected in stool samples. The patient was therefore treated with mebendazole, after which all symptoms permanently withdrew. About a month later laboratory examinations were repeated demonstrating increasing signs of renal damage. Kidney biopsy was performed, showing mesangioproliferative glomerulonephritis with crescents and IgA and C3 positive staining in the mesangium. Upon reviewing the clinical presentation, biochemically demonstrated progressive renal damage and biopsy results, the patient was diagnosed with HSP nephritis. CONCLUSION:The time course of the disease and present knowledge concerning the pathogenic mechanisms of HSP suggest that Strongyloides stercoralis infection could have caused HSP in the presented patient, which was complicated by nephritis.
Henoch-Schönlein purpura with intussusception and hematochezia in an adult: A case report.
Cui Xiao-Han,Liu Hao,Fu Li,Zhang Chao,Wang Xu-Dong
RATIONALE:Henoch-Schönlein purpura (HSP) is a common disease in children. However, HSP with intussusception and intestinal obstruction has a low morbidity in children and is occasionally seen in adults. Herein, a rare adult case of HSP complicated with intussusception and hemafecia that was successfully treated with surgery is described. PATIENT CONCERNS:A 19-year-old Chinese man suffered from HSP combined with intussusception and intestinal obstruction and presented with vomiting, diarrhea, abdominal pain, and rash. DIAGNOSIS:Henoch-Schönlein purpura with intussusception and intestinal obstruction. INTERVENTIONS:The patient underwent an emergency laparotomy and manual reset. OUTCOME:The patient was discharged 7 days after surgery in stable condition. We followed the patient to the sixth month after surgery. This patient has no long-term complications after surgery. LESSONS:HSP with intussusception and intestinal obstruction is rarely seen in adults. Patients with HSP often present with abdominal pain as the first symptom, which is easily confused with other diseases. Once the HSP is diagnosed, surgery should be performed as soon as possible, provided there is no absolute contraindication.
Resolution of sinus bradycardia, high-grade heart block, and left ventricular systolic dysfunction with rituximab therapy in Henoch-Schonlein purpura.
Torosoff Mikhail,Breen Thomas,Balulad Sujata,Padala Santosh,Lyubarova Radmila,Tan Henry,Sidhu Mandeep
Internal medicine journal
Henoch-Schonlein purpura (HSP) is a rare, typically self-limited, multi-organ vasculitis. Cardiac involvement with HSP carries high morbidity and mortality, thus requiring early aggressive immunosuppressive therapy. We report a case of HSP complicated with acute systolic left ventricular (LV) dysfunction, symptomatic sinus bradycardia and high-grade atrio-ventricular (AV) heart block. Cyclophosphamide, a commonly used agent in HSP, was contraindicated due to the patient's presentation with acute renal failure. Treatment with monoclonal antibody rituximab and corticosteroids was initiated with an improvement in and resolution of LV systolic dysfunction, sinus bradycardia and AV block. We believe this is the first published report on rituximab treatment in HSP with cardiac involvement manifesting with severe LV systolic dysfunction, sinus bradycardia and high-grade AV block.
Henoch-Schönlein Purpura With Testicular Necrosis: Sonographic Findings at the Onset, During Treatment, and at Follow-up.
Zhao Lixia,Zheng Shuguang,Ma Xiaojun,Yan Weihua
Henoch-Schönlein purpura (HSP) with involvement of the testes is extremely rare. Here, we present a pediatric case of HSP involving testicular ischemic necrosis in an 8-year-old child. Ultrasonography plays an important role not only in the differential diagnosis at onset, but also in the assessment of treatment response and prognosis during treatment and at follow-up. In this case report, we present the sonographic images for the entire course of testicular involvement in HSP and reveal histopathologically the pathogeny as testicular autoimmune vasculitis caused by the deposition of immunoglobulin A-containing immune complexes in the testicular vessels.
Dapsone treatment is efficient against persistent cutaneous and gastrointestinal symptoms in children with Henoch-Schönlein purpura.
Volejnikova Jana,Horacek Jaroslav,Kopriva Frantisek
Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia
BACKGROUND:Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and by the deposition of IgA immune complexes. An association between HSP and atypical bacteria is uncommon in children. METHODS AND RESULTS:Here we report three cases of children, aged 5, 4 and 16 years, who were diagnosed with HSP associated with Mycoplasma pneumoniae or Chlamydia pneumoniae infection. In all presented cases, persistent cutaneous manifestations and abdominal pain were resistant to antibiotics and corticosteroids, but resolved during 48 h after the introduction of dapsone. No adverse effects of treatment were observed. CONCLUSION:Dapsone, a sulphone with an anti-inflammatory activity, showed remarkable therapeutic efficacy against rash and gastrointestinal symptoms in children with HSP. Its administration should be considered particularly in persistent cutaneous form of HSP.
Stenosing ureteritis in Henoch-Schönlein purpura: report of two cases.
Kasahara Katsuaki,Uemura Osamu,Nagai Takuhito,Yamakawa Satoshi,Nakano Masaru,Iwata Naoyuki
Pediatrics international : official journal of the Japan Pediatric Society
Stenosing ureteritis (SU), a rare complication of Henoch-Schönlein purpura (HSP), typically presents with severe symptoms. We report the cases of two HSP patients presenting with gross hematuria, blood clotting, and colicky flank pain, followed by purpura on the lower extremities. Early-stage ultrasonography indicated hydronephrosis, thickened renal pelvic mucous membrane, and ureteral dilatation (UD), suggesting HSP complicated with SU. After early SU treatment with prednisolone, kidney function, thickened renal pelvic mucous membrane, and UD progressively normalized and the pain gradually disappeared. Regular ultrasonography of HSP patients from the onset of gross hematuria can be useful to detect early SU and facilitate conservative therapy with prednisolone. Diagnosis of SU can be easily missed by assuming HSP nephritis, particularly owing to the non-specific symptoms. Common characteristics as well as treatment methods and prognosis of SU are given in the literature review.
Henoch-Schönlein purpura complicated by acalculous cholecystitis and intussusception, and following recurrence with appendicitis.
Özkaya Ahmet Kağan,Güler Ekrem,Çetinkaya Ahmet,Karakaya Ali Erdal,Göksügür Yalçın,Katı Ömer,Güler Ahmet Gökhan,Davutoğlu Mehmet
Paediatrics and international child health
Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. Gastro-intestinal involvement occurs in two-thirds of patients. The characteristic skin lesions generally precede abdominal symptoms or present concurrently. A 7-year-old boy presented with intussusception and acalculous cholecystitis and had a cholecystectomy. Two weeks later he was re-admitted with features typical of HSP which responded to corticosteroids. Eleven months later he presented with abdominal pain and recurrence of HSP and, at laparotomy, there was acute appendicitis. This is the first case of a child presenting with HSP complicated by acalculous cholecystitis.
Henoch-Schönlein Purpura Complicated by Hepatocellular Carcinoma.
Akizue Naoki,Suzuki Eiichiro,Yokoyama Masayuki,Inoue Masanori,Wakamatsu Toru,Saito Tomoko,Kusakabe Yuko,Ogasawara Sadahisa,Ooka Yoshihiko,Tawada Akinobu,Maru Yugo,Matsue Hiroyuki,Chiba Tetsuhiro
Internal medicine (Tokyo, Japan)
Although Henoch-Schönlein purpura (HSP) is known to be accompanied by malignancies, cases with hepatobiliary cancer are extremely rare. A 62-year-old man with palpable purpura rapidly extending to both lower legs was admitted to our hospital. He was undergoing follow-up for cirrhosis caused by chronic hepatitis B virus infection and hepatocellular carcinoma (HCC). He had renal dysfunction with hematuria and proteinuria and abdominal pain. Based on the clinical presentation and skin biopsy findings, he was diagnosed with HSP. The administration of steroids resulted in the rapid improvement of the patient's symptoms and he was discharged 12 days after admission.
A girl with Henoch Schönlein purpura associated with acute rheumatic fever and review of literature.
Aypar Ebru,Demirtaş Duygu,Aykan Hayrettin Hakan,Kara-Eroğlu Fehime,Düzova Ali
The Turkish journal of pediatrics
Aypar E, Demirtaş D, Aykan HH, Kara-Eroğlu F, Düzova A. A girl with Henoch Schönlein purpura associated with acute rheumatic fever and review of literature. Turk J Pediatr 2018; 60: 576-580. Henoch Schönlein purpura (HSP) with acute rheumatic fever (ARF) is a rare entity and only few cases have been reported so far. In all previously reported cases with HSP and ARF, patients initially presented with purpuric rash, arthralgia/arthritis, or abdominal pain and later diagnosed as ARF. We report an 11-year-old girl with features of both ARF and HSP. She initially presented with arthralgia and murmur. Echocardiography showed mild to moderate mitral regurgitation. Later, the clinical course was complicated by purpuric rash and abdominal pain. She was treated conservatively with IM penicillin, acetylsalicylic acid and oral prednisolone. Our patient is the first patient with HSP and ARF who initially presented with features of ARF. A review of literature revealed a limited number of cases of HSP associated with ARF (14 cases including the present case); and that the response to treatment in cases suffering from ARF associated with HSP was good; but one should also be aware of serious cardiac complications in HSP patients which may be fatal.
Henoch-Schonlein purpura without typical lesions, presenting with gastrointestinal manifestations and kidney involvement following influenza - A case report.
Park Chan Won,Lim In Seok,Yun Sin Weon,Chae Soo Ahn,Lee Na Mi,Yi Dae Yong
JPMA. The Journal of the Pakistan Medical Association
We report a case of Henoch-Schonlein purpura (HSP) presenting without typical skin lesion; atypical symptoms initially appeared following influenza infection. A 4-year-old girl with influenza presented with epigastric pain and vomiting. On physical examination, there was epigastric tenderness, but no other signs, such as skin rash. On the second day, she vomited blood 10 times. Ultrasonography indicated focal bowel wall thickening in the right upper quadrant. Esophagogastroduodenoscopy showed oedematous and purpuric mucosa in the gastric pylorus and duodenum. Steroid therapy was initiated, and symptoms improved, but microscopic haematuria persisted. Even in the absence of typical purpura, if any gastrointestinal symptoms are observed and HSP is suspected, aggressive diagnostic tools must be considered, including ultrasonography or endoscopy. With only a few reported cases of HSP associated with influenza infection, this is the first reported case with gastrointestinal involvement and renal impairment, but without typical skin lesions.
Warfarin-induced toxic epidermal necrolysis in combination therapy of Henoch-Schönlein purpura nephritis: a case report.
Kasahara Katsuaki,Gotoh Yoshimitsu,Kuroyanagi Yoshiyuki,Nagano China
BACKGROUND:Toxic epidermal necrolysis (TEN) is a rare life-threatening condition almost exclusively attributed to drugs. The main etiologic factors for TEN are sulphonamides, anticonvulsants, and antibiotics; however, there are no published reports of warfarin causing TEN. CASE PRESENTATION:We present the case of a 3-year-old patient who developed TEN while receiving treatment for Henoch-Schönlein purpura nephritis (HSPN). With multiple-drug therapy comprising prednisolone, mizoribine, dipyridamole, and warfarin, it is difficult to detect which drug is the causative agent. While in most cases, diagnosis of the causative drug is based on clinical history without a lymphocyte transformation test (LTT), we performed the test three times and identified the causative drug as warfarin at the late phase. We continued HSPN treatment without warfarin, and results showed good renal function without life-threatening complications. CONCLUSION:To our knowledge, this is the first report about TEN caused by warfarin. Repeated LTTs could be useful for identifying TEN-causative drugs even in the late phase.
Multicentric Castleman's disease associated with IgA vasculitis (Henoch-Schönlein purpura) responding well to tocilizumab: a case report.
Oshima Yoichi,Hoshino Junichi,Suwabe Tatsuya,Hayami Noriko,Yamanouchi Masayuki,Sekine Akinari,Ueno Toshiharu,Mizuno Hiroki,Yabuuchi Junko,Imafuku Aya,Kawada Masahiro,Hiramatsu Rikako,Hasegawa Eiko,Sawa Naoki,Takaichi Kenmei,Hayashi Nobukazu,Fujii Takeshi,Ubara Yoshifumi
A 41-year-old man was referred to our hospital for the evaluation of hypergammaglobulinemia (IgG 2898 mg/dL and IgA 587 mg/dL), inflammation (CRP 6.7 mg/dL and serum interleukin-6 (IL-6) 15.1 ng/L), and anemia (Hb 10.9 mg/dL). Castleman's disease (CD) was diagnosed by axillary lymph node biopsy. Five months later, painful purpura (multiple palpable 5 mm lesions) developed on his legs, gradually spreading to the upper limbs, thighs, and trunk, accompanied by arthralgia of the wrists, ankles, and knees. Skin biopsy revealed leukocytoclastic vasculitis with IgA deposits in dermal vessels. Accordingly, IgA vasculitis (Henoch-Schönlein purpura) was diagnosed. Tocilizumab (an anti-IL-6 receptor antibody) was administered intravenously at 8 mg/kg and treatment was repeated at monthly intervals. His purpura and clinical findings specific to CD improved rapidly. CD is well known to cause various skin lesions. The findings in this case indicate that overproduction of IL-6 contributes to IgA vasculitis (Henoch-Schönlein purpura) as well as to the pathogenesis of CD.
Henoch-Schönlein purpura in the third trimester of pregnancy.
Djakovic Ivka,Butorac Drazan,Vucicevic Zeljko,Kosec Vesna,Kuna Andrea Tesija,Lugović-Mihić Liborija
Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessels' vasculitis that affects the skin, intestines and kidneys. Pregnancy has been reported as an exacerbating factor. We present the case of a 24-year-old with HSP that occurred in the third trimester and lasted up to the end of the successful delivery. She had pruritic maculopapular exanthema on her legs. Biopsy of a cutaneous lesion was performed for histopathologic features and direct immunofluorescence (DIF) for the presence of perivascular IgA deposition. Histopathology of the cutaneous lesion confirmed leukocytoclastic vasculitis. A DIF examination of the skin lesion confirmed deposits of fibrinogen in the small blood vessel walls. Six weeks following delivery, the skin lesions almost completely disappeared. Control laboratory findings were normal. This case of HSP might have been primarily associated with a preceding respiratory infection but this should first be carefully investigated due to a possible severe immunological disease in the patient's background requiring special attention since nephrotic symptoms may occur.
Successful treatment of hemorrhagic bullous Henoch-Schonlein purpura with intravenous immunoglobulins.
Mauro Angela,Mauro Salvatore,Rega Roberto,Martemucci Luigi,Sottile Rita
Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis in childhood. There is no consensus about the management for isolated cutaneous manifestations in HSP. We describe a case of HSP presenting with severe skin lesions that did not respond to standard therapy with corticosteroids. The 11-year-old child was treated with intravenous immunoglobulins, which induced rapid and persistent resolution of symptomatology.
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report.
Zhao Hui,Huang Wen-Hui,Huang Jun-Yue,Lu Shou-Yan,Yang Ya-Hong,Ma Zhi-Gang
Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas
Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.
Hemorrhagic bullous lesions in Henoch-Schönlein purpura: a case report and review of the literature.
Su Hung-Wen,Chen Chiu-Yu,Chiou Yee-Hsuan
BACKGROUND:Henoch-Schönlein purpura (HSP) is a common vasculitis in childhood characterized by purpura, arthritis, abdominal pain and renal involvement. However, bullous HSP is a rare cutaneous manifestation, and a few cases have been reported. CASE PRESENTATION:Herein, we report a 15-year-old male with bullous HSP who presented with severe abdominal pain and hemorrhagic bullous lesions over his lower extremities. He was treated with corticosteroid, after which the symptoms improved dramatically. No recurrence was noted after follow-up, though scarring was present. We also reviewed the literature related to bullous HSP and identified 39 cases, most of whom were treated with corticosteroids. CONCLUSION:Clinicians should be aware of the atypical types of HSP, including bullous HSP. Most patients with bullous HSP have a good prognosis.
Henoch-Schönlein purpura nephritis occurring postpartum in a patient with anti-PL-7 anti-synthetase syndrome.
Nagai Kojiro,Kishi Jun,Morizumi Shun,Minakuchi Jun,Bando Yoshimi,Nishioka Yasuhiko,Doi Toshio
A 37-year-old pregnant woman developed purpura which was subsequently diagnosed as Henoch-Schönlein purpura (HSP). After childbirth, the patient developed proteinuria and hematuria. Further examination revealed that the HSP nephritis (HSPN) was associated with anti-threonyl-tRNA synthetase anti-synthetase syndrome. The onset of HSPN during pregnancy or after childbirth is rare. Moreover, to our knowledge, this is the first case to describe renal involvement in anti-synthetase syndrome.
Henoch-Schonlein purpura, post-streptococcal glomerulonephritis and acute rheumatic carditis after Group A β-haemolytic streptococcal infection.
Arslansoyu Çamlar Seçil,Soylu Alper,Akil İpek,Ünlü Mehtat,Coşkun Şenol,Ertan Pelin,Kavukçu Salih
Paediatrics and international child health
Besides association with acute rheumatic fever (ARF) and acute glomerulonephritis (APSGN), in up to 40% of cases, Group A β-haemolytic streptococcal (GABHS) infections are also implicated as a trigger for Henoch-Schonlein purpura (HSP). A 7-year-old girl with GABHS throat infection who developed HSP, APSGN and rheumatic carditis is reported. She presented with palpable purpura and arthritis in both ankles and later developed carditis characterised by mitral/aortic regurgitation and glomerulonephritis characterised by mixed nephritic/nephrotic syndrome. She had a raised anti-streptolysin titre (ASOT), blood urea nitrogen and creatinine and hypocomplementaemia (C), and renal biopsy demonstrated endocapillary and extracapillary proliferative glomerulonephritis with crescents. Immunofluorescence microscopy demonstrated a 'full house' of immunoglobulin and complement, viz. IgA + 2, IgG + 3, IgM + 2, Cc + 1, Clq + 2 with predominantly IgG deposition. One week earlier, her 4-year-old sister had presented to another hospital with HSP complicated by microscopic haematuria, nephrotic-range proteinuria and gastro-intestinal involvement, and with raised ASOT and low C levels. Although HSP has been associated with either ARF or APSGN, this is the first case of a child with HSP, ARF and APSGN in combination.