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Abstract
Background Cutaneous Lupus Erythematosus (CLE) is a heterogeneous auto-inflammatory skin disease, that is to a great extent driven by type I and III interferon (IFN). Histology of skin biopsies plays an important role in the diagnostic confirmation of CLE. Unfortunately, no specific histological marker for CLE is available. In this study, we tested the diagnostic potential of immunostaining with Myxovirus resistance protein A (MxA), which is tightly induced by type I and type III IFN, in CLE skin biopsies.
Methods 178 skin biopsy specimens were collected from the local pathology database. Various skin conditions were selected, provided that clinical diagnosis matched with histological diagnosis. Skin sections were incubated with anti-MxA (R&D systems, AF7946). Consecutively, rabbit anti goat-HRP conjugate (Dako, 0449) was added and sections were stained with diaminobenzidine. The expression of MxA was scored semi-quantitatively.
Results MxA staining was strongly positive in 90.3% of lesional CLE skin sections (except lupus tumidus) and had a negative predictive value of 94%. The same MxA expression pattern was found in dermatomyositis, which is also an IFN-driven autoimmune disease. In some conditions, like perniosis and graft versus host disease, high expression could be found, but this was less consistent compared to CLE. Most other inflammatory skin diseases did show no or a low expression of MxA. (See figure 1).
MXA expression in all analyzed skin diseases (number of biospsies)
Conclusion MxA is strongly expressed in CLE skin with a high negative predictive value and is thus useful as additional diagnostic histological marker, expectedly resulting in restriction of misdiagnosis and treatment delay.