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Updated: Aug 13, 2026
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Rupert Waterhouse and Carl Friderichsen: adrenal apoplexy
J Varon1, K Chen, G L Sternbach
1Pulmonary and Critical Care Section, Baylor College of Medicine, The Methodist Hospital, Houston, Texas 77030, USA.
Abstract:
The Waterhouse-Friderichsen (WFS) syndrome, also known as purpura fulminans, is described as acute hemorrhagic necrosis of the adrenal glands and is most often caused by meningococcal infection. This clinical entity is more frequently seen in the pediatric than the adult population and is associated with a high morbidity and mortality. The initial presenting complaints for patients with the WFS usually include a diversity of nonspecific, vague symptoms such as cough, dizziness, headache, sore throat, chills, rigors, weakness, malaise, restlessness, apprehension, myalgias, arthralgias, and fever. These symptoms are usually abrupt in their onset. Petechiae are present in approximately 50-60% of patients. The clinical diagnosis of WFS may be relatively straightforward or extremely challenging. Patients who appear in the initial and nontoxic-appearing stage without any skin lesions may be difficult to distinguish from a benign viral illness. When a patient presents with fever and petechiae, WFS must be considered, even when the patient has a non-toxic appearance. Due to the rapid progression and often devastating consequences, therapy should be instituted as soon as the diagnosis is suspected.
Insights
Waterhouse-Friderichsen (WFS) syndrome, or purpura fulminans, causes acute adrenal gland necrosis, often from meningococcal infection. Early recognition of vague symptoms and petechiae is crucial for prompt treatment in this pediatric-leaning, high-mortality condition.
Area of Science:
- Pediatric Infectious Diseases
- Endocrinology
- Critical Care Medicine
Background:
- Waterhouse-Friderichsen (WFS) syndrome, also known as purpura fulminans, is a life-threatening condition characterized by acute hemorrhagic necrosis of the adrenal glands.
- It is most commonly caused by Neisseria meningitidis infection and predominantly affects the pediatric population, carrying high rates of morbidity and mortality.
- Initial symptoms are often nonspecific and vague, including fever, malaise, and respiratory or musculoskeletal complaints, making early diagnosis challenging.
Discussion:
- The clinical presentation of WFS can range from subtle, non-toxic appearing illness to rapidly progressing purpura fulminans.
- The presence of fever and petechiae should raise suspicion for WFS, even in the absence of a toxic appearance.
- Delayed diagnosis significantly worsens patient outcomes due to the rapid and severe nature of the disease.
Key Insights:
- WFS diagnosis requires a high index of suspicion, especially in children presenting with fever and petechiae.
- Nonspecific initial symptoms can mimic benign viral illnesses, complicating early recognition.
- Prompt initiation of therapy upon suspicion is critical to improve survival rates.
Outlook:
- Further research into earlier diagnostic markers and targeted therapies for WFS is warranted.
- Improved awareness among healthcare providers regarding the diverse presentations of WFS can facilitate timely intervention.
- Enhanced understanding of the pathophysiology may lead to novel treatment strategies for purpura fulminans.
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