Henoch-Schonlein Purpura is IgA mediated small vessel vasculitis. The most commonly affected organs are skin, gastrointestinal system and joints [1]. It is common in children aged between 3-10 years with male predominance with 1.2-1.8: 1 male/female ratio. The disease is characterized by palpable purpura in the dependent body parts like lower limbs and buttock, bowel angina along with hematuria/proteinuria and arthralgia/arthritis [2]. The etiologies proposed are infections, drugs or environmental exposure. The disease is self-limiting in most cases, but can cause renal complications including renal failure, hypertension and stroke. The symptoms abdominal and joint symptoms usually get resolved with NSAIDs and steroids. Steroids if started early has better clinical outcome with good prognosis [3].

Case

An 11 years old female child developmentally normal immunized for age presented with pain abdomen in the OPD of a regional health center with no fever, vomiting, loose stool, constipation and her systemic examination was unremarkable. She was started on symptomatic treatment. On day 2 of illness she developed rashes on bilateral lower limbs which were progressive and child reviewed in OPD with history of drug induced rashes and persistent pain abdomen. However, she was not having headache, fever, arthralgia, blood in stools and high colored urine. There was no recent history of infection or vaccination. Examination revealed raised pinkish-purple palpable purpura with mild skin edema (Figure 1).

Figure 1: Female child with palpable purpura in both lower limbe

On blood investigation she had normal platelet count, normal coagulation profile. She had proteinuria without hematuria. Ultrasound abdomen didn’t show intussusception or bowel wall edema or ischemia. She was diagnoses with Henoch Schonlein Purpura (HSP) and started on oral Prednisolone in view of persistent abdominal symptoms and nephritic range proteinuria. On follow up after one week the lower limb rashes got decreased (Figure 2). The treatment was continued. for another one week followed by tapering in next week. Child was asymptomatic on follow up after completion of treatment.

Figure 2: Same child after one week of Prednisolone treatment shows resolving rash.

HSP is the most common vasculitis of the children. Males are affected twice as common as females, however case discussed here was female child. The exact etiology and pathogenesis is not established but is attributed to different triggers like bacterial (mostly beta hemolytic streptococcus) and viral infections, vaccines, drugs and environmental exposures [4]. The immune complexes developed following antigen- antibody interaction get deposited in small vessels like capillaries, arterioles or venules. The complement mediated neutrophilic aggregation cause inflammation and vasculitis. The disease shows seasonal variation being common during autumn and winter season. The case described here presented during late November month and she didn’t have any history of proposed triggers. 

The clinical presentation of HSP includes classical tetrad of rashes, arthralgia, abdominal pain and renal involvement. Rashes are palpable and non-blanching type on dependent body parts. The joint involvement is in the form of pain and swelling of the joints mostly on knees and ankles. The abdominal features include vomiting and pain. There can be intestinal bleeds causing blood in stools. Renal involvement is in the form of microscopic hematuria and albuminuria [5]. The case discussed had abdominal pain, palpable purpura and proteinuria. There was no joint involvement. The disease is mostly self-limiting except in renal involvement where it can progress to renal failure. Prednisolone is the most commonly used steroid for the treatment of the disease [6]. Rare but morbid complications of HSP include intussusception, gastrointestinal bleed, stroke and cardiac involvement. Patients should be followed up with urine analysis for at least six months for renal complications [7].

1. Jennette, J.C .et al. “2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.” Arthritis & Rheumatism, vol. 65, no. 1, 2013, pp. 1–11.

2. Yang, Y.H., H.H. Yu, and B.L. Chiang. “The Diagnosis and Classification of Henoch–Schönlein Purpura: An Updated Review.” Autoimmunity Reviews, vol. 13, nos. 4–5, 2014, pp. 355–358.

3. Tizard, E.J., and M.J. Hamilton-Ayres. “Henoch–Schönlein Purpura.” Archives of Disease in Childhood: Education and Practice Edition, vol. 93, no. 1, 2008, pp. 1–8.

4. Saulsbury, F.T. “Clinical Update: Henoch–Schönlein Purpura.” The Lancet, vol. 369, no. 9566, 2007, pp. 976–978.

5. Roberts, P.F.et al. “Henoch–Schönlein Purpura: A Review Article.” Southern Medical Journal, vol. 100, no. 8, 2007, pp. 821–824.

6. Calvo-Rio, V.et al. “Henoch–Schönlein Purpura in Northern Spain: Clinical Spectrum of the Disease in 417 Patients from a Single Center.” Medicine, vol. 93, no. 2, 2014, pp. 106–113.

7. Narchi, H. “Risk of Long-Term Renal Impairment and Duration of Follow-Up Recommended for Henoch–Schönlein Purpura with Normal or Minimal Urinary Findings: A Systematic Review.” Archives of Disease in Childhood, vol. 90, no. 9, 2005, pp. 916–920.