Authors:Dr. Jerin K. John, Dr. Suma Balan (Pediatric Rheumatology,Dr. Sabin George (Pediatric Rheumatology), Dr. C. Jayakumar
A seven year-old boy, presented with a 6-day history of acute-onset diffuse abdominal pain, occasional vomiting, generalized body aches, and joint pain with swelling, in the feet and ankles. The mother reported reddish rashes over the both legs and thighs and one bloody vomitus.
The child was vaccinated for age, with no significant past medical history.
On admission, the child was afebrile, hemo dynamically stable, and appeared well-nourished.
On examination, palpable purpura was noted over the bilateral legs and thighs, swelling in the ankles and feet, and diffuse abdominal tenderness without guarding or rebound tenderness.
No organomegaly was observed.
Investigations included a skin biopsy, which showed basket weave keratin in the epidermis, hyalinization in the dermis, swollen endothelial cells, extravasated blood cells, leucocytoclasia, and a perivascular infiltrate of lymphocytes, eosinophils, and histiocytes. Immunofluorescence revealed IgA deposition in the walls of small blood vessels, confirming leukocytoclastic vasculitis.
Initial abdominal ultrasound revealed multiple small bowel loops with thickened and edematous walls, maximum thickness 3.6 mm in the right iliac fossa, inflamed adjacent mesentery, minimal interbowel free fluid, and mild right pleural effusion.
A follow-up ultrasound showed marked circumferential bowel wall edema, most pronounced in the umbilical region, with a maximum thickness of 6 mm, along with mild ascites that had progressed since the prior study.
No intussusception or other complications were noted.
Laboratory findings included hemoglobin of 11.8 g/dL, WBC count of 12.3 × 10⁹/L, platelet count of 330,000/µL, and an elevated CRP of 18 mg/L.
Urinalysis showed trace proteinuria without hematuria.
Cryoprecipitate (60 mL) was given empirically in view of suspected Factor XIII deficiency, and the child was treated with intravenous methylprednisolone.
Differential Diagnosis
• Intussusception: Ruled out imaging, as there was no evidence of telescoping bowel loops.
• Infectious Enterocolitis: Excluded based on the absence of systemic signs of infection, negative stool cultures, and the presence of palpable purpura.
• Inflammatory Bowel Disease (IBD): Considered due to abdominal pain and bowel wall edema but less likely given the acute onset and absence of chronic gastrointestinal symptoms or family history of IBD.
• Systemic Vasculitides (e.g., Kawasaki Disease, Polyarteritis Nodosa): Distinguished from HSP clinical features and biopsy findings specific to leukocytoclastic vasculitis with IgA deposition.
DiscussionHenoch-Schönlein Purpura (HSP) is characterized IgA-mediated small vessel vasculitis, often involving the skin, joints, gastrointestinal tract, and kidneys. Gastrointestinal manifestations, present in 50–75% of cases, range from abdominal pain and bowel wall edema to life-threatening complications such as intussusception or perforation.
The hallmark finding of bowel wall edema, evident in this case with maximum thickness reaching 6 mm, correlates with mucosal inflammation and ischemia caused vasculitis. While steroids remain controversial in HSP treatment, they are often employed in cases with severe abdominal pain or gastrointestinal bleeding.
Key differential diagnoses include intussusception, which was ruled out imaging, and infectious enterocolitis, excluded based on the absence of systemic signs of infection and negative stool cultures.
This case underscores the importance of recognizinggastrointestinal involvement in HSP, as delayed intervention may lead to significant morbidity. Close monitoring is crucial for detecting complications and ensuring resolution of symptoms.