Dermatology MCQ - Viral Infections - Chronic active Epstein-Barr virus infection

A 22-year-old woman presents with a 6-month history of persistent fever, fatigue, and lymphadenopathy. She has had multiple episodes of tonsillitis and now has hepatosplenomegaly. Laboratory studies show elevated EBV viral load in her peripheral blood, positive anti-VCA IgG, but negative anti-EBNA antibodies. A peripheral smear shows atypical lymphocytes. Which of the following is the most likely diagnosis?

A) Chronic active Epstein-Barr virus infection
B) Acute infectious mononucleosis
C) Hodgkin lymphoma
D) Systemic lupus erythematosus
E) Cytomegalovirus infection

Correct Answer: A) Chronic active Epstein-Barr virus infection

Explanation

This presentation is highly suggestive of chronic active Epstein-Barr virus infection (CAEBV), a rare and severe complication of EBV.

Key Clinical Features of CAEBV:

• Prolonged Symptoms: Persistent or recurrent infectious mononucleosis-like symptoms (fever, lymphadenopathy, fatigue) lasting >6 months.

• Organ Involvement: Hepatosplenomegaly is common. Other manifestations can include hepatitis, pancytopenia, interstitial pneumonitis, and uveitis.

• Laboratory Findings:

  • High EBV viral load in peripheral blood (by PCR).

  • Serology: Typically shows positive anti-VCA (viral capsid antigen) IgG and negative anti-EBNA (Epstein-Barr nuclear antigen) antibodies, indicating an inability to control viral replication and establish latency.

  • Atypical lymphocytes on peripheral smear.

Why Not the Other Options?

• (B) Acute infectious mononucleosis: Symptoms typically resolve within 2-4 weeks. Persistence beyond 6 months is not characteristic.

• (C) Hodgkin lymphoma: Can present with fever, lymphadenopathy, and fatigue (B symptoms), but would not typically show such high EBV viral load or the specific serologic pattern. Diagnosis requires biopsy.

• (D) Systemic lupus erythematosus: An autoimmune disorder that can cause fever and fatigue, but it is associated with autoantibodies (e.g., ANA, anti-dsDNA), not elevated EBV viral load.

• (E) Cytomegalovirus infection: Can cause a mononucleosis-like illness, but symptoms are usually self-limited. CMV PCR would be positive, and EBV serology/viral load would not fit.

Management:

• Immunotherapy: No standard treatment; options include rituximab (anti-CD20) to reduce B cells and cytotoxic chemotherapy.

• Stem cell transplantation: The only curative option for severe cases.

• Supportive care: Management of organ-specific complications.

Prognosis:

• Often progressive with high mortality due to organ failure, hemophagocytic lymphohistiocytosis, or lymphoma.

• Early recognition and referral for specialized care are crucial.