case_images: PMC6433840_01
case_images: Patient, familial CVID-affected subjects, and control subject plasmas were screened for the presence of neutralizing anticytokine autoantibodies. (A) Anticytokine autoantibodies were quantified as a function of the fluorescence intensity using a Luminex-based assay. (B) IFN-alpha or IFN-omega induced phosphorylation of STAT1. Normal control peripheral blood mononuclear cells were incubated with subject or control plasma, and STAT1 phosphorylation was measured in unstimulated or stimulated conditions via flow cytometry.
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case_images: PMC6433840_01_fped-07-00061-g0001.jpg
case_images: F1
case_images: A Luminex-based assay of the patient's plasma [1:100 dilution, as described by Ding et al. ] identified the presence of autoantibodies against interferon-alpha (IFN-alpha) and interferon-omega (IFN-omega) (Figure 1A). The patient's plasma demonstrated neutralizing anti-IFN-alpha and IFN-omega antibodies which inhibited STAT1-phosphorylation in control cells, whereas control and parental plasma did not lead to inhibition (Figure 1B).
case_images: Herpes vegetans facial lesions at initial presentation.
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case_images: PMC6433840_01_fped-07-00061-g0002.jpg
case_images: F2
case_images: The plaques were exudative and distorted the nasal architecture (Figure 2).
case_images: Herpes vegetans facial lesions after 10 days of intravenous foscarnet treatment.
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case_images: PMC6433840_01_fped-07-00061-g0003.jpg
case_images: F3
case_images: Intravenous foscarnet therapy resulted in rapid improvement of her cutaneous eruption, including the associated pain, and systemic symptoms (Figure 3).
case_images: Facial lesions 2 months after presentation.
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case_images: F4
case_images: Slower, continued resolution occurred over several weeks (Figure 4).