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115R.A. Watts and D.G.I. Scott (eds.), Vasculitis in Clinical Practice,DOI: 10.1007/978-1-84996-247-6_12, Springer-Verlag London Limited 2010

Chapter 12

HenochSchnlein Purpura

12.1 Introduction

Schnlein and Henoch independently described a syndromein children comprising joint pains, purpuric cutaneous lesions,abdominal colic, and malaena. Schnleins description pre-

ceded the one by Henoch by about 40 years, and there-fore, it is historically more accurate to term the syndromeSchnleinHenoch purpura. William Heberden wrote abouta child with arthralgia, edema, abdominal pain, vomiting,malaena, and purpura over the legs in 1801, 36 years priorto Schnlein.

12.2 Definition and Classification

The Chapel Hill Consensus Conference on the nomenclatureof systemic vasculitis defined HenochSchnlein purpura asVasculitis with IgA-dominant immune deposits affectingsmall vessels (capillaries, venules, or arterioles). It typicallyinvolves the skin, gut, and glomeruli and is associated with

arthralgias or arthritis.1

The ACR (1990) classification criteria (Table 12.1) havebeen widely used in clinical studies, but have relatively poorsensitivity (87.1%) and specificity (87.7%).2 These criteriahave recently been modified for use in children by theEuropean League Against Rheumatism and the European

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116 Chapter 12. HenochSchnlein Purpura

Pediatric Rheumatology Society (Table 12.2).3There are novalidated diagnostic criteria.

12.3 Epidemiology

HenochSchnlein purpura is a predominantly childhoodvasculitic syndrome. It is the most common vasculitis in chil-dren and can occur from 6 months of age onward. Most of thechildren are less than 10 years of age. There is no obviousgender predisposition. The incidence of HenochSchnlein

Table 12.1 ACR classification criteria for HenochSchnlein purpura.

Palpable purpura Slightly elevated purpuric rash over one or more areas of the

skin not related to thrombocytopaenia

Bowel angina Diffuse abdominal pain worse after meals, or bowel ischemia,

usually bloody diarrhea

Age at onset

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11712.5 Clinical Features

purpura in adults is lower than in children, and in the UK, theincidence is 20.4/100,000 children,4 and 1.3/100,000 adults

over the age of 16 years.5

12.4 Etiology

The etiology of HSP is unknown; however, there often appearsto be a triggering upper respiratory infection especially in chil-dren. A number of drugs such as penicillin, erythromycin, and

nonsteroidal anti-inflammatory drugs have been implicatedas triggers.

12.5 Clinical Features

The classical features of HSP are a triad of rash, gastrointes-

tinal upset, and joint pain.

12.5.1 Cutaneous

The classical rash of HenochSchnlein purpura is usuallythe first sign of the disease6 (Figs. 12.1 and 12.2). It is an

erythematous papular rash which develops into a palpablepurpura. The usual distribution is on the dependent andpressure-bearing areas of the lower limbs and buttocks. Thepurpura may be preceded by an urticarial rash. The rash isusually symmetrical and does not blanch with pressure. Skinnecrosis may be seen in areas of severe cutaneous hemor-rhage, and is more common in adults.

12.5.2 Gastrointestinal

The rash can be accompanied with gastrointestinal mani-festations including abdominal pain, nausea, and vomiting.Abdominal pain is perhaps the second most frequent clinical

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Figure 12.1 Purpuric lesions on the legs of a patient with HenochSchnlein purpura. Note the lesions along the pressure line causedby the patients socks.

Figure 12.2 Purpuric lesions on the abdomen in a patient withHenochSchnlein purpura.

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11912.6 Laboratory Features

manifestation after the skin rash. It is frequently colicky, andthe severity may wax and wane in relation with new crops of

skin lesions. The pain is thought to be due to submucosalhemorrhages. These can produce GI bleeds which can mani-fest as hematemesis or malaena. Infrequently acute intussuss-ception has been observed in children.

12.5.3 Musculoskeletal

Arthralgia is a common manifestation and can precede thedevelopment of palpable purpura by several days. Joint painswith or without obvious synovitis usually involves the lowerlimbs. Arthralgia is usually transient and does not leave anylong-term sequelae.

12.5.4 Renal

Renal involvement includes a spectrum of severity extendingfrom asymptomatic hematuria and mild proteinuria to glom-erulonephritis accompanied by hypertension and elevatedserum creatinine. The most severe clinical manifestation isprobably the mixed nephritic/nephrotic picture with hematu-

ria, hypertension, elevated serum creatinine, severe protei-nuria, and hypoalbuminemia. The renal involvement ofHenochSchnlein purpura is perhaps the most commonmanifestation responsible for the chronicity of the disease.The frequency of renal involvement is proportionally muchhigher in adults (80%) than in children (33%).

12.6 Laboratory Features

The inflammatory markers are almost always elevated. Thefull blood count shows leucocytosis and thrombocytosis inthe acute phase of the disease. A low hemoglobin may be asign of a gastrointestinal bleed.

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12.6.1 Renal Function

Urine may be positive for RBCs, leucocytes, and protein.Microscopy may reveal red cell casts in the presence of glom-erulonephritis. Renal function should be quantified withGFR, together with an estimation of urine protein leak.

12.6.2 Immunology

The serum IgA levels may be increased. Serological tests suchas ANA, ANCA, RF, and anticardiolipin antibodies should allbe negative. A positive ANA or ANCA should prompt anassessment for systemic vasculitis or SLE.

12.6.3 Pathology

The cutaneous lesions of HSP show a small vessel vasculitiswith the involvement of the capillaries, post capillary venules,and nonmuscular arteries. Often there is leucocytoclasis(Fig. 12.3). The appearances are difficult to distinguish fromleucocytoclastic (hypersensitivity) vasculitis or other small

Figure 12.3 Skin biopsy showing leucocytoclasis in a patient withHenochSchnlein purpura.

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12112.9 Treatment

vessel vasculitis. IgA deposits are present in the skin lesionsof HSP and may be a distinguishing feature.

A renal biopsy should be considered in both children andadults, if there is significant proteinuria or hematuria whichpersists. In the kidney, the earliest lesion is a focal or diffuseproliferative glomerulonephritis. The appearances may beindistinguishable from IgA nephropathy. Immunofluorescencereveals diffuse mesangial IgA deposition.

12.7 Diagnosis

The diagnosis of HSP should be suspected in children andadults who have purpuric lesions involving the lower limbs,with or without arthralgia, abdominal pain, or renal involve-ment. The diagnosis is confirmed with the classical histopatho-logical picture. There is no absolute diagnostic test, and the

criteria in Tables 12.1and12.2are classification criteria usedfor academic purposes. Other conditions, which can mimic HSP,are drug-induced vasculitis, and infections (especially Neiserriameningitides) and idiopathic thrombocytopenic purpura.

12.8 Assessment of Disease Activity

Disease activity assessment is largely clinical. The inflamma-tory markers in acute phase reactions usually follow the wax-ing and waning of the rash. Children with renal involvementcan continue to have microscopic hematuria and mild protei-nuria long after the acute phase of the disease. This does notnecessarily need escalation of treatment.

12.9 Treatment

Most children and adults with HSP do not need any specifictreatment. Treatment with nonsteroidal anti-inflammatoryagents is usually sufficient for the arthralgia. This should beavoided in patients with a gastrointestinal bleed. There is noevidence for the use of glucocorticoid therapy. In children,

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several RCTs have shown that the routine use of glucocorti-coids does not alter the progression to severe nephritis or GI

involvement.7,8

The acute hypertension may need treatmentwith an antihypertensive. Patients with acute renal failure willneed renal supportive treatment. There are no RCTs to guidetherapy in patients with rapidly progressive or establishedglomerulonephritis; such patients should be treated with glu-cocorticoids and immunosuppressants.

Glucocorticoids are effective in reducing the severity ofabdominal pain and arthralgias. Intussusception should be

excluded first.

12.10 Prognosis

Most children and adults with HSP have a self-limiting diseasewithin 23 weeks. However, the disease has been known torelapse in about 50% of children and a small proportion ofthese patients go on to develop chronic vasculitic syndrome.The long-term morbidity and mortality of HSP patients arepredominantly related to the level of renal involvement.Children presenting with acute nephritic syndrome have a lessfavorable outcome with a long-term risk of chronic renal fail-ure, but those with a mixed nephritic/nephrotic presentationhave the worst long-term outcome with up to a third develop-

ing chronic renal failure. Overall,

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123References

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4. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidenceof Henoch-Schnlein purpura, Kawasaki disease, and rare vasculitides in

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7. Huber AM, King J, McKaine P, Klassen T, Pothos M. A randomised pla-cebo controlled trial of prednisolone in early Henoch-Schnlein purpura.

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