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Henoch-schonlein purpura-a case report and review of the literature.

Sohagia AB, Gunturu SG, Tong TR, Hertan HI - Gastroenterol Res Pract (2010)

Bottom Line: It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis.The prognosis depends upon the extent of renal involvement, which requires close followup.Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

View Article: PubMed Central - PubMed

Affiliation: Gastroenterology, Montefiore Medical Center, North Division, 600, E 233rd street, Bronx, NY 10466, USA.

ABSTRACT
We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

No MeSH data available.


Related in: MedlinePlus

Histopathology of HSP involvement in skin showing microabscess.
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Related In: Results  -  Collection


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fig6: Histopathology of HSP involvement in skin showing microabscess.

Mentions: A 16-year-old Hispanic male from Puerto Rico presented with fever and sore throat for 5 days. He was given oral penicillin by his primary care doctor. After a day, he developed an erythematous, nonpruritic rash which progressed proximally from both feet to thighs and upper extremities including palms and soles. Later the feet became swollen with moderately intense (7/10) burning pain, aggravated by ambulation. Review of systems is unremarkable except left ear pain. His significant comorbidities include asthma and gastrojejunostomy following a remote motor vehicle accident. On physical examination, there was pharyngeal erythema, petechiae on the soft palate, cervical lymphadenopathy, a nodular, and nontender, nonblanching purpuric rash involving both upper and lower extremities with nonpitting pedal edema (Figures 1 and 2). There was no truncal involvement. Two days later, the patient developed abdominal pain involving the right and left upper quadrant which was constant, colicky in nature, 8/10 in intensity, aggravated with meals, and associated with hematemesis and watery stools. Laboratory tests showed leukocytosis (WBC: 16,900/microL); Hb: 14 g/dL; Hct: 41.2%; BUN: 16 mg/dL; Serum Creatinine: 0.9 mg/dL; Urinalysis: no hematuria or proteinuria; ESR: 58 mm/Hr; CRP: 5.6 mg/dL; Antistreptolysin O titer: 823 IU/L; C3: 125 mg/dL; C4: 11 mg/dL; ANA: Negative; EBV-VCA IgM: Negative; Anti-HAV IgM: Negative; HbsAg: Negative; Anti-HBc IgG: Negative; Mono spot test: Negative; c-ANCA: 0.2 units; p-ANCA: 0.2 units; Stool for occult blood: positive. Esophagogastroduodenoscopy (Figure 3) showed multiple erosions in the duodenum, antrum, and the gastrojejunal anastomotic site. Histopathology (Figures 4 and 5) of the small bowel showed preserved villous architecture, and neutrophilic and eosinophilic infiltrates with leukocytoclastic vasculitis. Warthin-Starry stain was negative for Helicobacter pylori. There was no evidence of epithelioid granuloma. Colonoscopy showed erythema and inflammation in the terminal ileum and cecum. Cecal biopsy also showed leukocytoclastic vasculitis. Skin biopsy (Figure 6) showed pustular leukocytoclastic fibrinoid vasculitis with microabscess. The patient was diagnosed with Henoch-Schonlein purpura as per American College of Rheumatology and European League Against Rheumatism (EuLAR) and Pediatric Rheumatology Society (PReS) criteria. He was treated with intravenous fluids and kept nothing by mouth for 3 days with no enteral or parenteral nutrition. He was started on oral prednisone 20 mg twice a day, with resolution of his symptoms and a decrease in ESR and CRP. His diet was advanced to regular diet on the fourth hospital day.


Henoch-schonlein purpura-a case report and review of the literature.

Sohagia AB, Gunturu SG, Tong TR, Hertan HI - Gastroenterol Res Pract (2010)

Histopathology of HSP involvement in skin showing microabscess.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2874920&req=5

fig6: Histopathology of HSP involvement in skin showing microabscess.
Mentions: A 16-year-old Hispanic male from Puerto Rico presented with fever and sore throat for 5 days. He was given oral penicillin by his primary care doctor. After a day, he developed an erythematous, nonpruritic rash which progressed proximally from both feet to thighs and upper extremities including palms and soles. Later the feet became swollen with moderately intense (7/10) burning pain, aggravated by ambulation. Review of systems is unremarkable except left ear pain. His significant comorbidities include asthma and gastrojejunostomy following a remote motor vehicle accident. On physical examination, there was pharyngeal erythema, petechiae on the soft palate, cervical lymphadenopathy, a nodular, and nontender, nonblanching purpuric rash involving both upper and lower extremities with nonpitting pedal edema (Figures 1 and 2). There was no truncal involvement. Two days later, the patient developed abdominal pain involving the right and left upper quadrant which was constant, colicky in nature, 8/10 in intensity, aggravated with meals, and associated with hematemesis and watery stools. Laboratory tests showed leukocytosis (WBC: 16,900/microL); Hb: 14 g/dL; Hct: 41.2%; BUN: 16 mg/dL; Serum Creatinine: 0.9 mg/dL; Urinalysis: no hematuria or proteinuria; ESR: 58 mm/Hr; CRP: 5.6 mg/dL; Antistreptolysin O titer: 823 IU/L; C3: 125 mg/dL; C4: 11 mg/dL; ANA: Negative; EBV-VCA IgM: Negative; Anti-HAV IgM: Negative; HbsAg: Negative; Anti-HBc IgG: Negative; Mono spot test: Negative; c-ANCA: 0.2 units; p-ANCA: 0.2 units; Stool for occult blood: positive. Esophagogastroduodenoscopy (Figure 3) showed multiple erosions in the duodenum, antrum, and the gastrojejunal anastomotic site. Histopathology (Figures 4 and 5) of the small bowel showed preserved villous architecture, and neutrophilic and eosinophilic infiltrates with leukocytoclastic vasculitis. Warthin-Starry stain was negative for Helicobacter pylori. There was no evidence of epithelioid granuloma. Colonoscopy showed erythema and inflammation in the terminal ileum and cecum. Cecal biopsy also showed leukocytoclastic vasculitis. Skin biopsy (Figure 6) showed pustular leukocytoclastic fibrinoid vasculitis with microabscess. The patient was diagnosed with Henoch-Schonlein purpura as per American College of Rheumatology and European League Against Rheumatism (EuLAR) and Pediatric Rheumatology Society (PReS) criteria. He was treated with intravenous fluids and kept nothing by mouth for 3 days with no enteral or parenteral nutrition. He was started on oral prednisone 20 mg twice a day, with resolution of his symptoms and a decrease in ESR and CRP. His diet was advanced to regular diet on the fourth hospital day.

Bottom Line: It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis.The prognosis depends upon the extent of renal involvement, which requires close followup.Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

View Article: PubMed Central - PubMed

Affiliation: Gastroenterology, Montefiore Medical Center, North Division, 600, E 233rd street, Bronx, NY 10466, USA.

ABSTRACT
We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

No MeSH data available.


Related in: MedlinePlus