We encountered a rare case, for which we performed a case study in conjunction with a brief literature review. This was accepted for publication in the International Journal of Dermatology. https://onlinelibrary-wiley-com.eu1.proxy.openathens.net/doi/full/10.1111/ijd.16025

Urticarial vasculitis (UV) is a type of small vessel vasculitis which presents as painful, long-lasting urticarial wheals (>24 hours) with specific findings present on histopathology. UV is further categorized into hypocomplementemic (HUV) and normocomplementemic (NUV) subtypes based on lab-measured complement levels. Approximately 80% of cases are NUV, and 20% are HUV. Systemic involvement is more frequent in HUV. We report a case of NUV with systemic involvement and an initial poor response to treatment, highlighting this rare entity and the associated unmet medical need.

Some cases are drug-induced, and these can be reversed by discontinuing the offending agent. For non-drug-induced cases, no official treatment guidelines currently exist for this condition. However, generally accepted practice dictates that oral corticosteroids are the first-line treatment, with dapsone and hydroxychloroquine presenting alternative options. In some cases, treatment requires augmentation with the addition of an immunomodulatory medication as well. Some other medications are being looked at for as possible treatment options, but more clinical trials are necessary to demonstrate a better understanding of this pathology.