Digital Ulcers and Microvascular Abnormalities Presenting As the Initial Manifestations of Pre-scleroderma

The term Raynaud's phenomenon (RP) is used to describe complex symptoms related to vascular compromise, which are typically exacerbated by cold-induced vasoconstriction, emotional stress, or other sympathomimetic factors. In almost all patients with limited cutaneous systemic sclerosis (SSc), the first symptom is RP, often two to five years before any other symptom of scleroderma. The clinical course and severity of this disease are variable and highly fatal in some individuals, which has led to the development of strategies for timely diagnosis; hence, criteria for the very early diagnosis of systemic sclerosis have been established. Nevertheless, some individuals with this disease may lack classification findings (Raynaud's phenomenon, puffy fingers, and positive antinuclear antibodies), making their condition less apparent. This study reports a different clinical scenario involving a previously healthy 42-year-old woman with recent monophasic RP and digital ulcers in the absence of clinical or immunological manifestations of systemic autoimmune disease. The diagnosis of preclinical systemic sclerosis was made possible based on evidence of capillaroscopic abnormalities. The patient demonstrated favorable evolution following the initiation of treatment with hydroxychloroquine, a statin, and a calcium antagonist. Four years later, she had no recurrences of digital ulcers, a noticeable improvement in her RP, and no clinical features of systemic sclerosis, highlighting the paradigm of the natural history of scleroderma in its preclinical, clinical, advanced severe stages, and immunological abnormalities.