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Acute Esophageal Necrosis Presenting With Henoch-Schönlein Purpura.

Iorio N, Bernstein GR, Malik Z, Schey R - ACG Case Rep J (2015)

Bottom Line: Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus.AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk.To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Section of Gastroenterology, Temple University Hospital, Philadelphia, PA.

ABSTRACT
A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk. To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis.

No MeSH data available.


Related in: MedlinePlus

Palpable, purpuric rash on the patient's (A) right thigh and torso and (B) hand.
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Figure 2: Palpable, purpuric rash on the patient's (A) right thigh and torso and (B) hand.

Mentions: The skin rash spread from her hands in a centripetal manner to involve the chest and thorax during the first day of her hospitalization (Figure 2). Skin biopsy revealed perivascular neutrophil infiltration of the upper dermis with neutrophilic degeneration, fibrin exudation, destruction of the vascular wall, and intravascular thrombi, consistent with leukocytoclastic vasculitis. A kidney biopsy was also performed and demonstrated proliferative glomerulonephritis and advanced interstitial fibrosis and tubular injury, with IgA and C3 deposition. Serum rheumatoid factor was negative. IgA was elevated at 633 mg/dL (normal: 85–385 mg/dL) and IgM was reduced at 24 mg/dL (normal: 45–250 mg/dL), while IgG was normal. C4 was normal and C3 was reduced at 67.5 mg/dL (normal: 70–176 mg/dL). Based on these findings and both gastrointestinal and renal involvement, the patient was diagnosed with Henoch-Schönlein purpura (HSP). Treatment with intravenous methylprednisolone was initiated. Her rash rapidly improved; however, she did require hemodialysis for oliguric renal failure. The AEN was managed conservatively with proton pump inhibitors. She required parenteral nutrition until her oral intake improved. A repeat endoscopy 9 days later revealed a diffuse and circumferential, pale yellow fibrinous layer in the esophagus and slightly improved ischemic duodenitis in the second portion of the duodenum (Figure 3).


Acute Esophageal Necrosis Presenting With Henoch-Schönlein Purpura.

Iorio N, Bernstein GR, Malik Z, Schey R - ACG Case Rep J (2015)

Palpable, purpuric rash on the patient's (A) right thigh and torso and (B) hand.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4612748&req=5

Figure 2: Palpable, purpuric rash on the patient's (A) right thigh and torso and (B) hand.
Mentions: The skin rash spread from her hands in a centripetal manner to involve the chest and thorax during the first day of her hospitalization (Figure 2). Skin biopsy revealed perivascular neutrophil infiltration of the upper dermis with neutrophilic degeneration, fibrin exudation, destruction of the vascular wall, and intravascular thrombi, consistent with leukocytoclastic vasculitis. A kidney biopsy was also performed and demonstrated proliferative glomerulonephritis and advanced interstitial fibrosis and tubular injury, with IgA and C3 deposition. Serum rheumatoid factor was negative. IgA was elevated at 633 mg/dL (normal: 85–385 mg/dL) and IgM was reduced at 24 mg/dL (normal: 45–250 mg/dL), while IgG was normal. C4 was normal and C3 was reduced at 67.5 mg/dL (normal: 70–176 mg/dL). Based on these findings and both gastrointestinal and renal involvement, the patient was diagnosed with Henoch-Schönlein purpura (HSP). Treatment with intravenous methylprednisolone was initiated. Her rash rapidly improved; however, she did require hemodialysis for oliguric renal failure. The AEN was managed conservatively with proton pump inhibitors. She required parenteral nutrition until her oral intake improved. A repeat endoscopy 9 days later revealed a diffuse and circumferential, pale yellow fibrinous layer in the esophagus and slightly improved ischemic duodenitis in the second portion of the duodenum (Figure 3).

Bottom Line: Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus.AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk.To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Section of Gastroenterology, Temple University Hospital, Philadelphia, PA.

ABSTRACT
A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk. To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis.

No MeSH data available.


Related in: MedlinePlus