Henoch-Schonlein Purpura—A Case Report and Review of the Literature

We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

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ADULT ONSET HENOCH: SCHONLEIN PURPURA – AN UNUSUAL PRESENTATION

Asian Journal of Pharmaceutical and Clinical Research ◽

10.22159/ajpcr.2017.v10i10.20007 ◽

2017 ◽

Vol 10 (10) ◽

pp. 2

Author(s):

Srujith Chandrakanti ◽

Oshin Mantro ◽

Jagadeesan Mohanan ◽

Mervin Kayipirambil Soman ◽

Prasanna Karthik Suthakaran ◽

...

Keyword(s):

Rare Case ◽

Early Recognition ◽

Leukocytoclastic Vasculitis ◽

Renal Involvement ◽

Unusual Presentation ◽

Organ Involvement ◽

Adult Onset ◽

Henoch Schonlein Purpura ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch–Schonlein purpura (HSP) is a common leukocytoclastic vasculitis seen in children. However, it is uncommon in adults. HSP is characterized by the classic tetrad of non-thrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal, and renal involvement. We report a rare case of adult onset HSP with multi-organ involvement. Early recognition of multi-organ involvement is very important, especially in adults.

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Koebner phenomenon in leukocytoclastic vasculitis: A case report and an updated review of the literature

SAGE Open Medical Case Reports ◽

10.1177/2050313x19850353 ◽

2019 ◽

Vol 7 ◽

pp. 2050313X1985035

Author(s):

Farah Kassam ◽

Sabrina Nurmohamed ◽

Richard M Haber

Keyword(s):

Case Report ◽

Leukocytoclastic Vasculitis ◽

Immunoglobulin A ◽

Small Vessel Vasculitis ◽

Review Of The Literature ◽

Henoch Schonlein Purpura ◽

Koebner Phenomenon ◽

Vessel Injury ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Leukocytoclastic vasculitis is the most common form of cutaneous vasculitis. It is a neutrophilic small vessel vasculitis resulting from the deposition of circulating immune complexes. Henoch-Schonlein purpura is a systemic type of leukocytoclastic vasculitis, characterized by immunoglobulin A-mediated blood vessel injury. We present a case of Henoch-Schonlein purpura in an adult female manifesting with a vasculitic rash with Koebner phenomenon.

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Henoch-Schonlein purpura masquerading as vesiculobullous lesions in a child

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20205417 ◽

2020 ◽

Vol 8 (1) ◽

pp. 170

Author(s):

Rajeshwari N. ◽

Prahada J. ◽

Kala K.

Keyword(s):

Leukocytoclastic Vasculitis ◽

Lower Limbs ◽

Small Vessel Vasculitis ◽

Direct Immunofluorescence ◽

Henoch Schonlein Purpura ◽

Diagnostic Challenge ◽

Serum Iga ◽

Ulcerative Lesions ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis occurring commonly in children presenting with palpable non-thrombocytopenic purpura, arthralgia and abdominal pain. Haemorrhagic bullous and ulcerative lesions are rare in HSP in children and can be a diagnostic challenge. We present a case of 6-year-old boy child who presented with bullous purpuric lesions in lower limbs, arthralgia and increased serum IgA. Histopathology of skin lesion revealed leukocytoclastic vasculitis. However, the direct immunofluorescence was negative for IgA deposits.

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Henoch-Schönlein purpura in children: an updated review

Current Pediatric Reviews ◽

10.2174/1573396316666200508104708 ◽

2020 ◽

Vol 16 ◽

Author(s):

Alexander K. C. Leung ◽

Benjamin Barankin ◽

Kin Fon Leong

Keyword(s):

English Literature ◽

Renal Involvement ◽

Clinical Manifestations ◽

Current Evidence ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Diffuse Abdominal Pain ◽

Excellent Outcome ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Background: Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small-vessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the most common form of systemic vasculitis in children. Objective: To familiarize physicians with the etiopathogenesis, etiology, clinical manifestations, evaluation, and management of children with Henoch-Schönlein purpura. Methods : A PubMed search was conducted in January 2020 in Clinical Queries using the key terms “Henoch-Schönlein purpura” OR “IgA vasculitis” OR “anaphylactoid purpura”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. This paper is based on, but not limited to, the search results. Results: Globally, the incidence of HSP is 10 to 20 cases per 100,000 children per year. Approximately 90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to 7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence does not support universal treatment of HSP patients with corticosteroids. Oral corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage. Conclusions: Most cases of HSP have an excellent outcome, with renal involvement being the the most important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP children who have severe nephritis or renal involvement with proteinuria of greater than 3 months, an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury.

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Henoch-Schönlein purpura presenting with multiplex gastrointestinal manifestations and massive nephrotic syndrome in adulthood — a case report

Open Medicine ◽

10.2478/s11536-008-0060-8 ◽

2008 ◽

Vol 3 (4) ◽

pp. 521-524

Author(s):

Farkas Klaudia ◽

Molnár Tamás ◽

Nagy Ferenc ◽

Tiszlavicz László ◽

Németh István ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Renal Involvement ◽

Gastrointestinal Symptoms ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Intravenous Cyclophosphamide ◽

Rapid Improvement ◽

Gastrointestinal Manifestations ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

AbstractHenoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis mainly affecting children. We report a case of a 49-year-old woman with severe gastrointestinal and renal involvement of HSP. Endoscopy revealed more characteristic findings in the terminal ileum than in the gastric antrum. Histological examinations of the biopsy samples from the ileum, antrum, skin and kidney confirmed the diagnosis of HSP. Parenteral corticosteroid therapy led to a rapid improvement of the gastrointestinal symptoms, but because of the excessive proteinuria intravenous cyclophosphamide therapy had to be introduced.

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Henoch-Schönlein Purpura Nephritis in Children: A Review

Bangladesh Journal of Child Health ◽

10.3329/bjch.v38i1.20024 ◽

2014 ◽

Vol 38 (1) ◽

pp. 24-31

Author(s):

Golam Muin Uddin ◽

Afroza Begum

Keyword(s):

Leukocytoclastic Vasculitis ◽

Treatment Options ◽

Renal Involvement ◽

Henoch Schonlein Purpura ◽

Multiple Organs ◽

Systemic Disorder ◽

Stage Renal Disease ◽

End Stage ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic disorder which involves multiple organs including kidney. The characteristic pathology is leukocytoclastic vasculitis of the capillaries with deposition of IgA immune complexes. The principal cause of morbidity and mortality in this disease is due to renal involvement. Approximately half of the children with HSP develop nephritis (HSPN) within 4-6 weeks of initial presentation and HSPN is a major cause of end stage renal disease (ESRD) in children. So, it is important to be updated about aetio-pathogensis, clinico-pathological aspects and prognostic factors of nephritis related to Henoch Schonlein purpura. The most important is to identify the appropriate treatment options for HSPN. So, herein review has been done on the different aspects of HSPN including treatment. DOI: http://dx.doi.org/10.3329/bjch.v38i1.20024 Bangladesh J Child Health 2014; VOL 38 (1) : 24-31

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