Henoch–Schönlein purpura

Published on 02/04/2015 by admin

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Last modified 22/04/2025

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154 Henoch–Schönlein purpura

Instruction

Look at this patient’s legs.

Diagnosis

This patient has purpuric rash over the legs and buttocks with renal involvement (lesions) caused by Henoch-Schönlein purpura (aetiology). I would like to know his 24-h urine output and levels of urea and electrolytes to determine renal function (functional status).

Advanced-level questions

What do you know about Henoch–Schönlein purpura?

Henoch–Schönlein or anaphylactoid purpura is a distinct, self-limiting vasculitis that occurs in children and young adults, with a peak incidence in the first two decades of life. It is a disorder characterized by non-thrombocytopenic purpura, arthralgia, abdominal pain and glomerular nephritis. It is the result of circulating immunoglobulin (Ig) A-containing immune complexes. It usually lasts between 1 and 6 weeks and subsides without sequelae if renal involvement is mild. The presence of IgG indicates a worse prognosis. Adults are more likely to develop renal involvement (Lancet 1992;339:280). In biopsy specimens obtained from the skin of patients with Schönlein–Henoch purpura, the dermal vessels frequently contain IgA deposits.

What investigations would you like to do?

• Examine the urine for haematuria and proteinuria

• Antinuclear factor

• Venereal Disease Research Laboratory Test

• Skin biopsy to detect arteriolar and capillary vasculitis.

What is the differential diagnosis?

• Drug-induced purpura

• Gonococcal arthralgia

• Keratoderma blenorrhagicum

• Secondary syphilis.

How would you classify vasculitis clinically?

• Purpuric disorders: Henoch–Schönlein purpura, leukocytic vasculitis

• Microscopic polyarteritis: polyarteritis nodosa

• Aortic type: Takayasu disease, polyarteritis nodosa (in the latter, the subclavian artery is spared)

• Pulmonary type: Wegener’s granulomatosus, Churg–Strauss syndrome.

How would you treat this patient?

• First line: rest, reduced activity and leg elevation

• First-line oral therapy: antihistamines and NSAIDS

• Second-line therapy: colchicine dapson

• Chronic or severe disease may require systemic immunosuppressants

• Patients with IgA nephropathy with protein excretion >500 mg/day, should be treated with an ACE inhibitor and/or an angiotensin receptor blocker. Patients with IgA nephropathy, increased urinary protein excretion (1.0–3.5 g daily) and plasma creatinine concentrations of ≤133 µmol/l (≤15 mg/l) may benefit from a 6-month course of steroid treatment (Lancet 1999;353:883–7). Steroids, plasma exchange, immunoglobulins and cytotoxic agents have all been used in complicated cases and there are no data from controlled trials (Lancet 2000;356:562).

What is the prognosis of Ig A nephropathy?

IgA nephropathy tends to progress slowly and only about half the patients develop end-stage renal disease within 25 years. Factors that predict an accelerated course are persistent proteinura, persistent microscopic haematuria, elevated serum creatinine at the time of diagnosis, poorly controlled hypertension and extensive glomerulosclerosis or interstitial fibrosis on renal biopsy.

What is the the difference between primary Ig A nephropathy and Henoch–Schönlein purpura?

IgA nephropathy (or Bergers disease) was first described by Berger and Hinglais as recently as 1968 (J Urol Nephrol (Paris) 1968;74:694–5) and is now generally accepted to be the most common form of primary glomerulonephritis (N Eng J Med 2002;347:738–48). There are two conditions that should be distinguished from primary IgA nephropathy: Henoch–Schönlein purpura and thin glomerular basement membrane disease. Thin glomerular basement membrane disease is a common condition that occurs more often in female patients and has a benign clinical course in that end-stage renal disease does not develop. Its diagnosis is established by electron microscopy, where glomerular capillary loops can be shown to have diffusely attenuated basement membranes.

What are the other cutaneous manifestations of immune complex-mediated small vasculitis?

Vesicles, pustules, superficial ulcerations, urticaria, splinter haemorrhages, hyperpigmentation (Fig. 154.2).

Fig. 154.2 Immune complex-mediated small vessel vasculitis. (A) Vesicles. (B) Pustules. (C) Superficial ulcerations. (D) Urticaria. (E) Splinter hemorrhages. (F) Hyperpigmentation.

(With permission from Firestein et al. 2008.)

What are the different types of immune complex-mediated vasculitis?

• Henoch–Schönlein purpura

• Cutaneous leukocytoclastic angiitis

• Mixed cryoglobulinaemia

• Connective tissue disease/rheumatoid vasculitis.

This disorder was first reported in the English literature by Willan in 1808.

Eduard Heinrich Henoch (1820–1910) qualified in Berlin, where he ran the neurology department; he studied under Schönlein and eventually became a paediatrician. In 1874 he reported the coexistence of purpura and GI haemorrhage.

Johannes Lucas Schönlein (1793–1864) was initially Professor of Medicine in Würzburg but, because of his liberal views, he was dismissed; he eventually became Professor of Medicine, first in Zurich and then in Berlin. In 1837 he reported the coexistence of purpura and arthritis.

Anthony Fauci is contemporary Director of the National Institute of Allergy and Infectious Diseases and Director of AIDS Research, NIH, Bethesda; his chief interests include immunological disorders including AIDS.

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