Pediatr Infect Vaccine.  2015 Dec;22(3):210-215. 10.14776/piv.2015.22.3.210.

Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus

• Noh JH
• Jeong DY
• Jeon IS
• Kim HM

• KMID: 2315688
• DOI: http://doi.org/10.14776/piv.2015.22.3.210

Abstract

Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.

Keyword

Macrophage activation syndrome; Herpes viral infection; Systemic lupus erythematosus

MeSH Terms

Anemia, Hemolytic
Aspartate Aminotransferases
Bone Marrow
Exanthema
Female
Ferritins
Fever
Hepatomegaly
Humans
Kaposi Varicelliform Eruption
L-Lactate Dehydrogenase
Lupus Erythematosus, Systemic*
Lymphohistiocytosis, Hemophagocytic
Macrophage Activation Syndrome*
Macrophage Activation*
Macrophages*
Oral Ulcer
Pancytopenia
Rheumatic Diseases
Steroids
Aspartate Aminotransferases
Ferritins
L-Lactate Dehydrogenase
Steroids

Figure

• 

  Fig. 1. Multiple diffuse erythematous papules, pustules, and patches with edema were on the face and scalp.

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  Fig. 2. Skin biopsy of erythematous macule on her face. In the microscopic view, mild spongiosis, focal basal vacuolar degeneration, dermal edema and minimal perivenular and perifollicular lymphocytic infiltration were observed, as signs of inflammatory changes in the skin lesions.

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  Fig. 3. Clinical course of the patient which shows the fever and laboratory changes as patient undergoes treatment. ∗Fever refers peak body temperature of the day. Taspartate aminotransferase, white blood cell, hospital day.

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