Waterhouse-Friderichsen syndrome in dengue fever

A 3-month-old male infant presented to the Emergency Department with a history of generalised rashes, starting with tiny red spots and then coalescing into islands of red rashes within 4 hours. Before that, he had a history of fever for 1 week associated with vomiting and reduced oral intake. Upon arrival at the hospital, upon admission, the infant was initially managed with supportive care, including intravenous fluids to maintain hydration and antipyretics to control fever. Due to the severity of the condition, resuscitation efforts included aggressive fluid management to counteract shock and administration of intravenous ceftriaxone, a broad-spectrum antibiotic to cover potential bacterial infections. Continuous monitoring of vital signs was critical, and as the condition worsened, inotropic support was employed to stabilise blood pressure and ensure adequate perfusion to vital organs. However, the corticosteroids were not given in this case due to unsuspected of the possibility of adrenal insufficiency. Unfortunately, the child succumbed to death about an hour later despite resuscitation.

The medicolegal autopsy was performed due to the unexplained death of the infant. Despite intensive treatment, the cause of death was not apparent, prompting a complete autopsy to determine the underlying factors contributing to the fatal outcome. Autopsy findings showed a generalised purpura that spares some distal extremal areas, mimicking meningococcemia rashes (Figure 1). Multiple petechiae were seen at the conjunctivae, extremities, and back. Adrenal gland haemorrhage was present, consistent with the features of Waterhouse-Friderichsen syndrome (Figure 2).

Figure 1 Generalized purpura that spares some distal extremal areas. Informed consent was taken from the decedent’s father.

Figure 2 Adrenal glands haemorrhage.

Comprehensive bacteriological and viral studies were conducted in this case, including blood cultures; tissue culture from the lungs, spleen, and lymph nodes; cerebrospinal fluid culture and antigen detection for Hemophilus influenza, Streptococcus group B, Streptococcus pneumoniae, E. coli K1, and Neisseria meningitidis; and serological analysis for viruses such as dengue, measles, mumps, rubella, parvovirus and varicella, and polymerase chain reaction (PCR) for standard pathogens such as respiratory and dengue viruses. The investigation results showed only serological evidence of primary dengue infection. The bacteriology and other viral studies were negative. Histology sections from the adrenal glands showed extensive areas of haemorrhage, corroborating the gross findings of the adrenal glands (Figure 3). The brain tissue histological specimen showed no evidence of meningitis or encephalitis. The toxicological analysis was sent to the Chemistry Department to rule out any evidence of poisoning, and the results were negative for any poison and toxic substances.

Figure 3 Histology slide of the adrenal glands showed an extensive haemorrhage (H&E staining, ×100). H&E, hematoxylin and eosin.

The presentation of this dengue case in this infant is considered rare, as Waterhouse-Friderichsen syndrome itself is rare in dengue fever. The implications of this rarity are significant as it presents unique challenges in diagnosis, even in the postmortem setting. Diagnosing dengue fever in such a young infant can be complicated by the nonspecific nature of symptoms, often mistaken for other common infant illnesses. The rarity also poses challenges in pediatric care, as standard treatment protocols might not account for such rare presentations, necessitating more specialised and vigilant care.

Acknowledgments

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Footnote

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Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Informed consent was obtained from the deceased’s father for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

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