Henoch-Schonlein purpura (HSP) is an autoimmune condition also referred to as anaphylactoid purpura. It is a self-limited systemic, IgA-mediated small vasculitis. It is non-granulomatous, autoimmune complex, with multi-organ involvement, and most commonly in children. Its etiology is unclear but is associated with upper respiratory tract infections, drugs, foods, insect bites, vaccinations, tumors (non-small cell lung cancer, prostate cancer, and hematological malignancies), alpha-1-antitrypsin deficiency, and Familial Mediterranean Fever.
Case Stem We present a 17 years old caucasian male with 3 weeks history of itchy erythematous, palpable pruritic rash which progressed proximally from both feet to thighs and upper extremities. He went to an outlying facility and was given steroids for a few days. Symptoms subsided temporary. Later the patient developed peri-umbilical abdominal pain which was colicky in nature, 8/10 in intensity, associated with vomiting, bloody diarrhea, joint pain and fever. Review of systems was unremarkable otherwise. Abdominal examination showed mild tenderness in the peri-umbilical area, non-blanching palpable purpuric rash involving both upper and lower extremities.
Laboratory tests showed leukocytosis, high CRP and ESR.
Normal Hb, Hct, BUN, Serum Creatinine, Urinalysis, C3 and C4.
Anti-HAV IgM: Negative; HbsAg: Negative; Anti-HBc IgG Negative.
Colonoscopy showed pancolitis. Abdominal ultrasonography detected increased bowel wall thickness, skin biopsy was not specific because it was taken after 24 hrs of once of rash.
The patient was diagnosed with HSP as per the American College of Rheumatology and European League Against Rheumatism (EuLAR) as well as Pediatric Rheumatology Society (PReS) criteria.
He was treated with intravenous fluids and was abstained from food and liquids. He was started on oral prednisone. He recovered well and was discharged with a follow up appointment.
Conclusion Although HSP patients mostly range from 4–7 years old it can still presents in adolescent age group. Non-thrombocytopenic palpable purpura with multi-organ involvement (gastrointestinal, skin and joints) should make one consider the diagnosis. skin biopsy should be taken less than 24 hrs from the presentation because in more chronic lesions, vessel damage leads to nonspecific leakage of all isotypes of immunoglobulin⇓.
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