A previously healthy 11-year-old boy presents with purpuric macules and papules symmetrically distributed on his buttocks and upper and lower extremities. What’s the diagnosis?
A previously healthy 11-year-old boy presents with purpuric macules and papules symmetrically distributed on his buttocks and upper and lower extremities. He had been admitted to the pediatric inpatient service after his mother brought him to the pediatric emergency department with diffuse abdominal pain; nausea; and persistent, nonbloody, and nonbilious vomiting several times a day for 2 days. His pain was localized to his epigastrium and was described as sharp, persistent, 6 of 10 in severity, and nonradiating. The macules and papules had developed 4 days after admission.
During the initial examination, he appeared mildly dehydrated, was afebrile, and had a heart rate of 112 beats per minute, respiratory rate of 18 breaths per minute, and blood pressure of 112/82 mm Hg. His weight was 40.2 kg. He had no relevant family history.
Laboratory results showed serum amylase and urine amylase double the standard value. All other blood work results, including leukocyte count, platelet count, and C-reactive protein, were within limits. Viral screen and screens for autoantibodies were negative. On ultrasonography, normal echogenicity with mild pancreatic enlargement was found. He received a diagnosis of acute pancreatitis and was admitted for supportive medical therapy with intravenous hydration, pain control, nutritional supplementation, and bowel rest.
What’s the cutaneous diagnosis?
This patient’s nonthrombocytopenic, nontender, palpable purpura was clinically diagnosed as Henoch-Schönlein purpura. A skin biopsy confirmed the diagnosis. The patient was kept under observation and supportive therapy for 1 week and discharged for outpatient follow-up after a reduction of symptoms.
The incidence of acute pancreatitis (AP) is estimated at 3.6 to 13.2 per 100,000 children per year. It is an important differential for children with acute abdominal symptoms. Pediatric cases are estimated to account for less than 1% of all cases of acute necrotizing pancreatitis, which is characterized by inf lammation of the pancreas.
Diagnosis is made by reviewing the symptoms (usually vomiting and abdominal pain), pancreatic lipase and amylase levels, and imaging. Ultrasound is a useful noninvasive modality to assess the pancreas, but CT scanning is superior for early diagnosis, especially when ultrasound findings are nonspecific. Magnetic resonance cholangiopancreatography is essential in patients where no cause is found, as this may show underlying anatomic anomalies.
Cutaneous manifestations in pancreatic diseases
Skin clues occur in 3% of patients with pancreatitis and may predict the severity of pancreatitis and the development of complications. Several such cutaneous manifestations have been described in both inf lammatory and neoplastic conditions of the pancreas.
Henoch–Schönlein purpura (HSP) is the most common vasculitis in children and has been associated with acute pancreatitis. The mean age of presentation of HSP is 6 years, with most cases in children younger than 13 years. It is associated with IgA deposition in vessel walls and usually presents as 2-mm to 7-mm nonblanching papules, often with a surrounding collar of purpura and joint pain. Gastrointestinal symptoms may precede the purpura by up to 2 weeks in approximately 20% of children with their first HSP episode, with the rash commonly occurring approximately 1 week (average 6.6 days) after the onset of abdominal pain. Renal involvement, also called HSP nephritis, has been reported to occur in 20% to 80% of the patients with HSP and is clinically manifested by microscopic or macroscopic hematuria, proteinuria, nephrotic syndrome, and reduced kidney function. Renal involvement in HSP is a major cause of chronic renal failure in children, and the long-term principal prognostic determinant depends on the severity of renal damage. Although the cause is unknown, HSP is often preceded by an acute infectious illness and has a seasonal pattern (nonsummer months), providing strong evidence for an infectious trigger.
Endoscopic, radiological, and ultrasound studies are used in the diagnosis of GI complications in HSP. Recent studies demonstrate that fecal calprotectin can be elevated in acute phases of gastrointestinal involvement in HSP. Therefore, it has been proposed as a marker for early diagnosis of GI involvement in HSP.
Treatment is symptomatic and may include mild analgesics such as acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) for joint pain and fever. However, NSAIDs should be avoided if the patient has gastrointestinal or renal manifestations, as they have been shown to aggravate these symptoms. All patients should undergo blood pressure mea-surement and urinalysis at the time of diagnosis followed by periodic urinalysis for at least 6 months.
Cullen sign, Grey-Turner sign, Fox sign
It is thought that 1 or more of the signs may be present in 1% to 3% of patients with acute pancreatitis. Cullen sign is described as superficial edema with bruising of the subcutaneous fatty tissue around the periumbilical region. Grey-Turner sign is bruising of the lateral aspect of the abdominal wall or the flanks. In Fox sign, bruising is seen over the proximal thigh.
The discoloration around the periumbilical region can vary in color from green/yellow to purple but varies according to the stage of breakdown of the red blood cells. Pancreatic enzymes have been suggested as a mechanism leading to the discoloration of the abdominal wall adipose tissue.
This occurs in 2% to 3% of patients with pancreatic disease. It often presents as purple-red tender nodules or plaques on the extremities, trunk, and buttocks.
Acanthosis nigricans is a common skin condition characterized by hyperpigmented plaques, with papillomatous hyperkeratosis, giving a velvety texture, distributed symmetrically on intertriginous sites. It is usually associated with a benign form of insulin resistance in obesity.
Necrolytic migratory erythema is a cutaneous sign of glucagonoma that is characterized by itchy, painful, ring-shaped red patches that blister and crust over.
Livedo reticularis is characterized by mottled cyanotic discoloration of the skin, and it has a particular network pattern. Walzel sign, which is livedo reticularis on the abdomen, chest and/or thighs, is believed to be associated with trypsin-induced damage to the subcutaneous venous network seen in acute and chronic pancreatitis.
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