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Henoch-Schönlein Purpura

Schonlein-Henoch Purpura

Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated vasculitis that affects small vessels.


Presentation

The onset of purpura, abdominal pain, and arthritis may occur in any order. It is important to remember that abdominal pain and arthritis are not universally present in patients. The timing of symptoms may vary from within days or insidious over a period of weeks [6]. Children will almost always have complaints of purpura and gastrointestinal (GI) symptoms in up to 70% cases. Adults will often complain of symptoms pertaining to skin and joints.

Palpable purpura

  • Palpable purpura , a manifestation of vascular inflammation, is the hallmark presentation of HSP presents regardless of age group. 
  •  Purpura is defined as cutaneous hemorrhage measuring greater than 3mm but less than 1mm in diameter. It does not blanch under pressure [9]. Purpura starts as erythematous macules and papules or urticarial wheals that develop into a non-pruritic rash. The rash then mature into multiple petechiae that eventually coalesce into purpura. The purpuric lesions turn from red to purple to rust-colored over a span of approximately 10 days as hemoglobin products breakdown [9]. Over time, the purpura may coalesce and ulcerate.
  •  The appearance of purpura occurs due to extravasation of blood into the skin and mucous membranes. The most common sites are dependent areas that are frequently subjected to pressure. Some examples of these areas are the lower extremities, belt line, and buttocks. The extensor surfaces of extremities are more involved than the flexor area [10]. 

Polyarthralgia

  • Polyarthralgia occurs in joints with underlying immune complex deposition. The knee and ankle joints are common sites of joint pain [3] [4].
  • Multiple joint pains, accompanied by erythema, swelling, and tenderness are often encountered even without the presence of frank arthritis. 
  • The joint pains are transient and do not produce deformities. It may present prior to purpuric rash in 15 to 25 percent of patients [3] [4]. 

GI symptoms

  • GI symptoms are commonly encountered in the pediatric patients. They usually complain of colicky abdominal pain. Other GI symptoms such as nausea, vomiting, diarrhea or constipation accompany the abdominal discomfort. Some cases include presence of blood or mucus in the stool. 
  • In very rare instances, extravasation of blood from small vessels of the GI tract may become severe that it forms a hematoma. Mural hematoma can serve as the nidus for an unwanted complication – intussusception. Intussusception is a condition that involves bowel telescoping.

Renal involvement

  • 10- 50% of HSP patients can develop renal complications. Renal involvement is usually incidental when a patient undergoes urinalysis as patients are often asymptomatic. Renal pathology is often detected with laboratory results of proteinuria, microscopic hematuria, and microscopic casts in majority of patients. Hematuria may be present in some individuals.
  • Renal involvement usually resolves spontaneously. It is very rare that progressive glomerulonephritis develops. Nonetheless, the course of renal disease can be very misleading; thus, it is prudent to advice patients for close follow-ups. 

Orchitis

  • Testicular and scrotal swelling is seen in up to 35 percent of boys with HSP [6]. 
  • Severe scrotal edema may cause compression of the spermatic cord and its testicular vessel contents. This cut off blood supply to the testes leading to ischemia in a condition called testicular torsion. Testicular torsion requires emergent surgical exploration [6].
Easy Bruising
  • There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums.[medical-dictionary.thefreedictionary.com]
Fever
  • Henoch Schönlein purpura (HSP) with acute rheumatic fever (ARF) is a rare entity and only few cases have been reported so far.[ncbi.nlm.nih.gov]
  • Ibuprofen is a nonsteroidal anti-inflammatory drug that is used widely in treating pain, fever, and inflammation. Its side effects are mainly due to acute renal impairment and gastric discomfort.[ncbi.nlm.nih.gov]
  • A 7-year-old girl was admitted to the hospital with abdominal pain, vomiting, and fever. Combining clinical manifestations with results of radiologic examinations, acute appendicitis was suspected and a laparotomy was considered.[ncbi.nlm.nih.gov]
  • The patient presented with fevers, fatigue, abdominal pain and renal failure and was found to have acute left-sided staphylococcal endocarditis. He subsequently developed small bowel perforation and purpuric rash.[ncbi.nlm.nih.gov]
  • We herein report the case of a 61-year-old Japanese cirrhotic patient who developed rat bite fever (RBF) and whose first presentation was serious clinical features mimicking those of Henoch-Schönlein purpura (HSP).[ncbi.nlm.nih.gov]
Rapidly Progressive Glomerulonephritis
  • In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed.[ncbi.nlm.nih.gov]
  • A kidney biopsy was performed and the presence of IgA deposits and cellular crescents indicated rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura (HSP).[ncbi.nlm.nih.gov]
  • Urinary findings deteriorated in three patients after the appearance of purpura, including one patient who developed rapidly progressive glomerulonephritis. Renal biopsy findings worsened in two patients.[ncbi.nlm.nih.gov]
  • Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation.[ncbi.nlm.nih.gov]
  • Patients with rapidly progressive glomerulonephritis (RPGN) have been anecdotally reported to benefit from intensive plasma exchange combined with cytotoxic drugs.[symptoma.com]
Abdominal Pain
  • Her abdominal pain gradually improved over the next week, and a repeat computed tomography scan showed significant improvement of the small bowel wall thickening and pneumatosis. The purpuric rash improved, and her abdominal pain resolved.[ncbi.nlm.nih.gov]
  • Most cases of Henoch-Schönlein purpura with severe abdominal pain are treated with low-dose steroids and intravenous immunoglobulin. A 15-year-old Korean girl complained of severe abdominal pain and delayed-onset purpura on admission.[ncbi.nlm.nih.gov]
  • Abdominal pain in patients with HSP may herald a serious gastrointestinal problem also in adult patients.[ncbi.nlm.nih.gov]
  • The abdominal pain and purpura resolved at discharge and there were no recurrences in the subsequent 3-, 6-, and 12-month follow-ups.[ncbi.nlm.nih.gov]
  • The diagnosis of HSP is difficult to establish, especially when the purpura occurs after gastrointestinal involvement; thus, abdominal pain should not be ignored and HSP should be considered.[ncbi.nlm.nih.gov]
Vomiting
  • 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.[ncbi.nlm.nih.gov]
  • Three weeks later, he presented again with loss of appetite and vomiting before developing abdominal pain with pyrexia of 38.5 C and rigors. On examination, he was hypotensive with a distended abdomen and a national early warning score of 6.[ncbi.nlm.nih.gov]
  • A 7-year-old girl was admitted to the hospital with abdominal pain, vomiting, and fever. Combining clinical manifestations with results of radiologic examinations, acute appendicitis was suspected and a laparotomy was considered.[ncbi.nlm.nih.gov]
  • We present the case of a 37-year-old male who presented with complaints of nausea, vomiting, abdominal pain, purpuric rash over lower extremities, and migratory polyarthralgia five days after being treated with antibiotics for bronchitis.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Nausea
  • We present the case of a 37-year-old male who presented with complaints of nausea, vomiting, abdominal pain, purpuric rash over lower extremities, and migratory polyarthralgia five days after being treated with antibiotics for bronchitis.[ncbi.nlm.nih.gov]
  • Some of the complicaitons of Henoch-Schönlein are blood in the stool and nausea from the abdominal pain. A combination of the purpura, joint pain and abdominal pain is usually enough to confirm a diagnosis of Henoch-Schönlein.[house.wikia.com]
  • Abdominal pain Joint pain Abnormal urine (may have no symptoms) Diarrhea, sometimes bloody Hives or angioedema Nausea and vomiting Swelling and pain in the scrotum of boys Headache The health care provider will look at your body and look at your skin.[nlm.nih.gov]
  • A group of symptoms are often found in patients and can include: gastrointestinal complaints (such as abdominal pain, nausea, vomiting, and bleeding), kidney disease which can present with blood in the urine, joint pains (frequently in the larger joints[aocd.org]
  • Signs and symptoms usually begin suddenly (and progress over days) and may include purple-colored spots on the skin (purpura); joint pain; and gastrointestinal problems such as abdominal pain, nausea, bloody stools, and rarely, severe complications requiring[rarediseases.info.nih.gov]
Severe Abdominal Pain
  • Most cases of Henoch-Schönlein purpura with severe abdominal pain are treated with low-dose steroids and intravenous immunoglobulin. A 15-year-old Korean girl complained of severe abdominal pain and delayed-onset purpura on admission.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Severe abdominal pain with hematemesis developed, along with an increase in liver aminotransferase levels. Henoch–Schönlein purpura was suspected.[nejm.org]
  • If your child stops eating or drinking, or develops severe abdominal pain or kidney problems, hospitalization might be necessary. Outlook Most children with HSP fully recover within a month and have no long-term problems.[rchsd.org]
  • These include: Hematuria Blood-staining of stools Arthralgia or arthritis Severe abdominal pain Testicular pain or edema Further Reading Where are the Kidneys and Liver Located? Renal Neoplasms Anatomy of the Kidney[news-medical.net]
Loss of Appetite
  • Three weeks later, he presented again with loss of appetite and vomiting before developing abdominal pain with pyrexia of 38.5 C and rigors. On examination, he was hypotensive with a distended abdomen and a national early warning score of 6.[ncbi.nlm.nih.gov]
  • Intestines : The main symptom is abdominal pain, but symptoms can also include loss of appetite, diarrhea, vomiting and occasional blood in stools. Rarely, patients develop an abnormal bowel folding called intussusception, which can be serious.[my.clevelandclinic.org]
  • The symptoms of HSP are often preceded by cold and flu symptoms, such as fever , headache , nausea , loss of appetite, or diarrhea .[my.clevelandclinic.org]
  • In more than half of people with HSP, inflammation of the gastrointestinal tract may cause pain or cramping; it may also lead to loss of appetite, vomiting , diarrhea , and occasionally blood in the stool.[webmd.com]
Hypertension
  • Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness.[ncbi.nlm.nih.gov]
  • A 52-year-old man presented with a purpuric rash affecting his legs and hypertension. He was diagnosed with Henoch-Schönlein purpura and discharged with non-steroidal anti-inflammatory drugs.[ncbi.nlm.nih.gov]
  • Furthermore, neither patient showed any adverse effects, such as hypertension, encephalopathy, or chronic nephrotoxicity, to the CyA treatment.In conclusion, these results suggest that CyA may be a safe and effective treatment for steroid-resistant severe[ncbi.nlm.nih.gov]
  • Further investigations may be required if the diagnosis is unclear, abdominal symptoms are severe or where there is evidence of significant renal involvement (hypertension, macroscopic haematuria or proteinuria) Most cases are self-limiting and require[rch.org.au]
Purpura
  • The presence of purpura facilitates the diagnosis of Henoch-Schönlein purpura at the onset of associated symptoms, whereas the absence of purpura makes the diagnosis challenging.[ncbi.nlm.nih.gov]
  • Although HSPN usually develops after the appearance of anaphylactoid purpura, we have encountered patients whose renal symptoms preceded purpura.[ncbi.nlm.nih.gov]
  • Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon.[ncbi.nlm.nih.gov]
  • The presence of purpura is a compulsory criteria for the diagnosis of Henoch-Schönlein purpura (HSP).[ncbi.nlm.nih.gov]
  • A normal platelet count differentiates Henoch-Schönlein purpura from thrombocytopenic purpura. Skin biopsy may show a leukocytoclastic vasculitis. Treatment There is no specific treatment for Henoch-Schönlein purpura.[aafp.org]
Palpable Purpura
  • The criteria selected by a classification tree method were similar: palpable purpura, age less than or equal to 20 years at disease onset, biopsy showing granulocytes around arterioles or venules, and gastrointestinal bleeding.[ncbi.nlm.nih.gov]
  • In both lower extremities, there were numerous palpable purpura and hemmorrhagic bullae. In light of clinical findings, laboratory tests and skin biopsy are requested.[ncbi.nlm.nih.gov]
  • Besides the most common clinical manifestations such as palpable purpura, arthralgia, abdominal pain and renal disease, it can have urological manifestations.[ncbi.nlm.nih.gov]
  • Henoch-Schonlein purpura (HSP) may present in a variety of ways, most commonly with joint pain or the distinctive palpable purpura.[ncbi.nlm.nih.gov]
  • Henoch-Schönlein purpura (HSP) is a systemic vasculitis that is common in the pediatric population and often presents with the classical triad of palpable purpura, arthralgia, and abdominal pain.[ncbi.nlm.nih.gov]
Petechiae
  • A 64-year-old man was admitted to the hospital with petechiae and palpable purpura in lower limbs and abdominal pain for about 1 month.[ncbi.nlm.nih.gov]
  • Patients with this disorder typically present with palpable purpura or petechia associated with one or more of the following signs and symptoms: abdominal pain, arthritis/arthralgias, and nephritis.[ucdavis.pure.elsevier.com]
  • This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP.[ncbi.nlm.nih.gov]
  • It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.[mountsinai.org]
  • Henoch-Schönlein purpura Search terms Child, Dermatology, Gastroenterology, HSP, Hematuria, Henoch-Schonlein purpura, Henoch-Schönlein purpura, Nephrology, Paediatrics, Purpura, Schoenlein-Henoch, Rheumatology, arthritis, haematuria, nephritis, papule, petechia[ebm-guidelines.com]
Urticaria
  • Click here for example image of HSP Links: aetiology pathogenesis clinical features treatment and prognosis complications urticaria[gpnotebook.co.uk]
  • Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement.[ebi.ac.uk]
  • A rash may start as hive-like spots ( urticaria ) or small raised red spots ( erythematous maculopapules ) on the legs and buttocks. Eventually these spots blend to form bigger areas of bruising ( purpura ) in the skin.[jamanetwork.com]
  • Henoch-Schönlein purpura a type of nonthrombocytopenic purpura, of unknown cause but thought to be due to a vasculitis; it is most often seen in children and is associated with clinical symptoms such as urticaria and erythema, arthritis and other joint[medical-dictionary.thefreedictionary.com]
Exanthema
  • 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.[ncbi.nlm.nih.gov]
Arthritis
  • This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP.[ncbi.nlm.nih.gov]
  • Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is characterized by non-thrombocytopenic purpura, abdominal pain, arthritis, and glomerulonephritis.[ncbi.nlm.nih.gov]
  • The dominant clinical features of HSP are cutaneous purpura (100%), arthritis (82%), abdominal pain (63%), gastrointestinal bleeding (33%), and nephritis (40%).[ncbi.nlm.nih.gov]
  • Henoch-Schönlein purpura is a common small-vessel vasculitis that presents in children as nonthrombocytopenic, nontender, and palpable purpura, arthritis/arthralgia, and abdominal pain.[ncbi.nlm.nih.gov]
  • Arthritis/arthralgia may be a risk factor according to the criteria of the American College of Rheumatology (1.41, 1.01-1.96).[ncbi.nlm.nih.gov]
Arthralgia
  • 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.[ncbi.nlm.nih.gov]
  • Besides the most common clinical manifestations such as palpable purpura, arthralgia, abdominal pain and renal disease, it can have urological manifestations.[ncbi.nlm.nih.gov]
  • Henoch-Schönlein purpura (HSP) is a systemic vasculitis that is common in the pediatric population and often presents with the classical triad of palpable purpura, arthralgia, and abdominal pain.[ncbi.nlm.nih.gov]
  • Henoch-Schönlein purpura is a common small-vessel vasculitis that presents in children as nonthrombocytopenic, nontender, and palpable purpura, arthritis/arthralgia, and abdominal pain.[ncbi.nlm.nih.gov]
  • The patient presented with a purpuric rash and arthralgia. During admission, he developed hematochezia and acute kidney injury.[ncbi.nlm.nih.gov]
Ankle Arthritis
  • This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP.[ncbi.nlm.nih.gov]
Hematuria
  • RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Henoch-Schonlein purpura is an immunoglobulin A (IgA)-immune complex mediated leukocytoclastic vasculitis that classically manifests with palpable purpura, abdominal pain, arthritis, and hematuria or proteinuria.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Macroscopic Hematuria
  • Most adults and children with HSP nephritis present with hematuria, including macroscopic hematuria, which is more commonly noted in children.[propath.com]

Workup

The diagnosis of Henoch-Schönlein purpura is often based on clinical signs and symptoms [1].

Complete blood count

Complement fixation test

  • Serum complement components are within normal levels.

IgA component

  • Secretory IgA levels are above the normal limits in approximately one-half of patients [1].

Skin biopsy

  • Skin biopsy specimen can be useful in confirming leukocytoclastic vasculitis with IgA and C3 deposition by immunofluorescence [1]. 

Renal biopsy

  • Nephrology referral is recommended with significant renal involvement.
  • Patients with severe renal disease should undergo renal biopsy. It serves as a definitive diagnostic tool. It also aids in prognostic information and guides therapy [7].

Hospitalization may be required in these cases:

  • Adequate outpatient monitoring is unavailable.
  • Dehydration, hemorrhage, or pain control requires meticulous management [1]. 
Abnormal Renal Function
  • renal function Macroscopic haematuria for 5 days Nephrotic syndrome Acute nephritic syndrome Persistent proteinuria UPCR 250mg/mmol for 4 weeks UPCR 100mg/mmol for 3 months UPCR 50mg/mmol for 6 months If there is no significant renal involvement plus[rch.org.au]
Staphylococcus Aureus
  • He was diagnosed with catheter-related infection by Staphylococcus aureus. After administration of antibiotic and steroid therapy, purpura disappeared and p-ANCA gradually became negative.[ncbi.nlm.nih.gov]
  • We report an elderly patient with the end-stage renal disease presented with diffuse palpable purpura while receiving IV vancomycin therapy for methicillin-resistant Staphylococcus aureus septicemia.[ncbi.nlm.nih.gov]

Treatment

The treatment of HSP is the same for adults and children.

Supportive treatment is the mainstay treatment. Most patients are able to recover completely without longstanding complications. Some cases do not even necessitate therapeutic intervention [7].

Various studies yield positive outcomes of the role of steroid intervention in management of HSP:

  • Early use of glucocorticoids is most beneficial to children with renal involvement or severe extra-renal symptoms such as colicky abdominal pain and polyarthralgia [8]. Steroid intervention may help in orchitis relief among young boys.
  • Oral prednisone should be started at 1 to 2 mg per kg daily and tapered according to clinical response. This has been used to treat moderate to severe abdominal and joint symptoms and tissue edema [1]. It can accelerate healing of HSP symptoms in children [11]. Corticosteroid use in children with HSP reduced the mean time to resolving abdominal pain. Moreover, the odds of developing persistent renal disease decrease [8].
  • Although prednisone does not provide adult renal disease prophylaxis, it was useful in treatment after onset of renal disease [8].

Patients with rapidly progressive glomerulonephritis (RPGN) have been anecdotally reported to benefit from intensive plasma exchange combined with cytotoxic drugs [1].

Prognosis

HSP cases resolve spontaneously in 94 percent of children and 89 percent of adults [6] [7] with excellent prognosis. Mortality is exceedingly rare and roughly 1–5% of children progress to end-stage renal disease [7].

Disease recurrences have been found in 10-40% of patient. This usually subsides within 3–6 months. Longer clinical course have been reported in patients with renal involvement [8].

Etiology

The list of common offenders is as follows:

Upper respiratory tract infection (URTI)

URTI is a common antecedent for HSP.

  • Group A streptococcus organsims are the most common cause of URTI. It has been found in cultures of more than 30% of children with Henoch-Schönlein nephritis.
  • Serum antistreptolysin-O titers (ASO) are more likely to be positive in patients with Henoch- Schönlein Purpura nephritis [2] [3].
  • ASO titers are indicative of a recent group A streptococcus infection in the last 45 days.

Drugs

  • Medications such as penicillin, insulin, and sulfa drugs are notorious for causing allergic reactions. However, people react to drugs differently. An individual can have an idiosyncratic reaction to any drug he/she takes.

Foods

  • Foods such as peanuts, chocolates, and seafoods are well-known for their role in allergic reactions.

Insect bites

  • Each individual reacts differently to an insect bite or sting. Most will have localized swelling but a few can be severely allergic to them and present with life-threatening reactions.

Immunizations

  • Vaccines contain large amount of proteins that may trigger severe adverse immunologic responses in certain persons.

Pollen

  • Pollen is infamous for causing seasonal allergies such as hay fever.

Other types of allergen

Epidemiology

About 10 to 22 per 100,000 persons are seen by physicians for HSP every year [3] [4].

HSP has a special predilection for school-age children.

  • Children with age range of 4-7 years old are most likely to suffer from HSP. Nonetheless, infants and young adults can also be affected.

Males are more affected than females by a slight margin.

  • The male-to-female ratio of patients with HSP is 1.5:1 [1].

The disease peaks during spring season.

  • Spring is the time of the year when pollen becomes abundant as trees and grass bloom. Pollen is a very-well known allergen that can trigger immunologic reactions. Some trees (i.e. Oak or Maple) and grass (i.e. Bermuda grass) are known allergy offenders too.
Sex distribution
Age distribution

Pathophysiology

The clear mechanism for development of HSP is not yet clearly understood. It is widely based on an assumption that deposition of immune complexes, such as immunoglobulin A (IgA), and granulocyte debris in the walls of small blood vessels cause extravasation of red blood cells into the dermis. Therefore, the most common HSP clinical presentations are petechiae and palpable purpura. Numerous antigens from allergens can elicit immunologic cascades and immune complex deposition.

The symptoms of patients depend on the location of immune complex depositions. When immune complexes occur in small vessels of the intestinal wall, gastrointestinal hemorrhage may develop. If the immune complexes successfully penetrate the renal mesangium, it may produce mild proliferative to severe crescentic glomerulonephritis [5].

Prevention

HSP is mainly immunologic. Avoiding known and potential allergens will help prevent acquiring HSP.

Summary

Henoch-Schönlein purpura (HSP) is a systemic vasculitis of the small blood vessels. It is also known as anaphylactoid or allergic purpura due to HSP’s association with an immunologic reaction. It is often secondary to allergens, streptococcal infections, insect stings, or drug administration. The disease most frequently occurs in the pediatric and young adult population.

Immune complex can burrow through the walls of small blood vessels, particularly in the skin, joints, intestines and kidneys. This often leads to a diffuse inflammation of the affected blood vessels. As a consequence, there is leakiness of affected blood vessels that is characterized by a distinct palpable purpura (most commonly seen over the buttocks and lower extremities), multiple joint pains, gastrointestinal (GI) signs and symptoms, and glomerulonephritis [1].

Patient Information

Overview

HSP is the body’s response to certain triggers such as allergens, pollen, medications, and infections. The body reacts by releasing allergy-fighting chemicals against the offender. Unfortunately, these chemicals deposit in the small blood vessels. This deposition produces holes and blood cells leak into the layer beneath the skin. The result of the leakiness is a cluster of symptoms collectively known as HSP.

Symptoms

Skin lesions

  • The trademark feature of HSP is a distinguishing elevated skin lesion called purpura.
  • The size of a purpura is more than 3mm in diameter with various colors of red, purple or rust. It can be best described as a big, palpable bruise although bruise and purpura are not the same.

Tummy ache

  • Tummy ache is common among kids. It occurs in more than 2/3 of children with HSP.Tummy upset is caused by leaking of small blood vessels in the digestive system. 
  • Tummy disturbances can be accompanied by loose bowel movements, constipation, and sensation of wanting to vomit.

Joint pains

  • Multiple joint pains are common among adults. It may be accompanied by swelling, redness, and pain upon touch.

Swelling of the testes

  • Swelling of the testes can be seen in 1/3 of young boys with HSP.
  • Severe pain in the testes should raise a concern for the possibility of testicular torsion, a surgical emergency.

Treatment

Treatment mainly focuses on providing symptom relief by giving pain killers, anti-inflammatory drugs, and adequate fluid intake. The disease is allergic in nature and heals on its own after 2-4 weeks [12] in 90% of cases.

Close monitoring and follow-up of patients is necessary in very few instances of kidney involvement. Referral to a nephrologist is also done to avoid further kidney complications.

Home remedy for symptom relief:

  • Acetaminophen may be used to relieve joint pains. NSAIDs are now discouraged because it may aggravate stomach ache and induce kidney injury.
  • Resting and elevation of affected extremities may prevent promulgation of purpura.
  • Recurrent purpura may be experienced with increased activity levels.
  • Elevation of extremities will decrease blood flow and immunologic components deposition in the affected areas.

References

Article

  1. Longo D, Kasper D, Jameson J, et al. Harrison’s Principles of Internal Medicine 18th ed. New York: McGraw-Hill; 2011.
  2. Saulsbury FT. Epidemiology of Henoch Schönlein purpura. Cleve Clin J Med. 2002; 69 : SII87-SII90.
  3. Masuda M, Nakanishi K, Yoshizawa N, Iijima K, Yoshikawa N. Group A streptococcal antigen in the glomeruli of children with Henoch-Schönlein nephritis. Am J Kidney Dis. 2003; 41: 366-370.
  4. Trapani S, Micheli A, Grisolia F, et al. Henoch-Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and a review of the literature. Semin Arthritis Rheum. 2005; 35: 143-153.
  5. Saulsbury FT. Henoch-Schönlein purpura. Curr Opin Rheumatol. 2001; 13: 35-40.
  6. Gedalia A. Henoch-Schönlein purpura. Curr Rheumatol Rep. 2004; 6: 195-202.
  7. Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis. 2006; 47(6): 993-1003. 
  8. Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006; 149:241-247.
  9. Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. 2007; 369: 976-978.
  10. Reamy B, Tammy L. Henoch-Schönlein purpura.Am Fam Physician. 2009; 80: 697-704
  11. Matucci- Cerinic M, Furst D, Fiorentino D.Skin Manifestations in Rheumatic Disease.New York: Springer New York.2014. 
  12. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics. 2007; 120: 1079-1087.

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Last updated: 2018-06-21 21:58