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

Abstract

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.

Keywords: immunosuppressive agents; nailfold videocapillaroscopy; raynaud's phenomenon; scleroderma; vasculopathy.

Figure 1. Bilateral Raynaud's phenomenon with vascular involvement.

(A) Monophasic Raynaud's phenomenon (redness) on the right index finger. (B) Marked periungual erythema. (C) Digital necrosis and ulceration on the right index fingertip.

Figure 2. Nailfold videocapillaroscopy showing a capillaroscopy pattern consistent with early scleroderma.

(A) Giant capillaries >50 µm in diameter (×200). (B) Capillary microhemorrhages (arrow) and giant capillaries >50 µm in diameter (×200).

Figure 3. Pre-scleroderma: diagnostic and therapeutic approach.

*Treatment includes hydroxychloroquine (200-400 mg daily), high-intensity statin, and calcium channel antagonist. The image is created by the author of this study.