Raynaud’s Phenomenon, Anticentromere Antibodies and Digital Necrosis (RACAND) Is a Distinct Clinical Syndrome From Systemic Sclerosis: A Case Report and Literature Review

The occurrence of digital necrosis and Raynaud's phenomenon with anticentromere (ACA) antibodies has been recognized as a rare clinical entity coined RACAND syndrome. Although these features occur in systemic sclerosis (SSc), RACAND syndrome lacks other features characteristic of SSc, such as sclerodactyly, skin thickening or organ involvement. Reports of this syndrome are scarce throughout the literature, with only 10 described cases of RACAND syndrome, but several more reports of digital gangrene associated with ACA lacking SSc features. In this article, we present the case of an 80-year-old woman who developed digital gangrene, with Raynaud's phenomenon and ACA in the absence of other signs and symptoms and no SSc features. The patient was diagnosed with RACAND syndrome and responded well to treatment with iloprost and moderate doses of prednisone. Additionally, a review of the available literature of similar cases is shown in order for clinicians to gain better insight of disease characteristics and possible treatment options, as there are no established treatment guidelines. The majority of patients were females of older age, mostly without medical history which would predispose them to a peripheral vasculopathy. Treatment including prostanoids might yield more promising results, however this requires further dedicated studies. We argue that RACAND syndrome is distinct from SSc and that the absence of other typical diagnostic features of SSc or other systemic autoimmune disease is necessary for diagnosis. Clinician awareness of this entity is needed in the treatment of digital necrosis, as well as further studies to determine the best treatment options.