Child Kidney Dis.  2019 Oct;23(2):128-133. 10.3339/jkspn.2019.23.2.128.

A Case of Henoch-Schönlein Purpura with Fulminant Complications and Its Long-term Outcome

• Lee DH
• LEE ES
• Hong J
• Park KH
• Pai KS

• KMID: 2464940
• DOI: http://doi.org/10.3339/jkspn.2019.23.2.128

Abstract

Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan (99mTc RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.

Keyword

HSP; Intestinal perforation; Nephritis; Pleural hemorrhage

MeSH Terms

Abdominal Pain
Adult
Arthritis
Biopsy
Cyclosporine
Diagnosis
Emergencies
Erythrocytes
Exanthema
Follow-Up Studies
Hemorrhage
Humans
Intestinal Perforation
Intestines
Intussusception
Kidney
Laparotomy
Lower Extremity
Nephritis
Purpura*
Skin
Systemic Vasculitis
Cyclosporine