Девушка с признаками синдрома Рейно: описание симптомов и методы диагностики

Slideshow - Slide A 20-year-old woman presents with episodic color changes of her fingertips triggered by cold exposure, including holding cold beverages. She reports that the episodes begin with pallor and a numb sensation, followed by a dusky or bluish discoloration, and then bright red discoloration upon rewarming of her fingers. These episodes are intermittent and have been occurring for several years. She notes mild discomfort during attacks but denies ulceration, persistent pain, or tissue breakdown. She has no history of autoimmune disease, vascular disorders, or prior similar conditions and is not taking any medications. On physical examination during an episode, there is sharply demarcated pallor of multiple fingertips (Figure). The involved digits are cool to the touch, and the findings are present in both hands. No ulceration or digital pitting is observed. There is no evidence of skin thickening, sclerodactyly, or telangiectasias. Nailfold capillary examination is unremarkable, without dilated or tortuous capillaries. Raynaud phenomenon is a vasospastic disorder characterized by episodic digital ischemia in response to cold exposure or emotional stress, presenting as biphasic or triphasic color changes of the digits, classically progressing from white (pallor) to blue (cyanosis) to red (hyperemia).1,2... Submit your diagnosis to see full explanation. Raynaud phenomenon is a vasospastic disorder characterized by episodic digital ischemia in response to cold exposure or emotional stress, presenting as biphasic or triphasic color changes of the digits, classically progressing from white (pallor) to blue (cyanosis) to red (hyperemia).1,2 The condition affects approximately 3% to 5% of the general population, with most cases representing primary Raynaud phenomenon.2 Clinically, attacks consist of well-demarcated color changes because of transient vasospasm and subsequent reperfusion.1,2 The fingers are most commonly involved, though toes and other acral sites may also be affected. In primary Raynaud phenomenon, involvement is typically symmetric with sparing of the thumbs.1 Severe cases, particularly secondary Raynaud phenomenon, may result in complications such as digital ulcers or tissue necrosis.1,3 Episodes are transient and often resolve within minutes after removal of the trigger. Diagnosis is clinical and based on history and direct observation of characteristic color changes. Additional evaluation may be performed to distinguish primary from secondary disease, including nailfold capillaroscopy and autoantibody testing. Normal nailfold capillaries and negative serologies support a primary process.1 Initial management focuses on nonpharmacologic measures, including cold avoidance, smoking cessation, and avoiding vasoconstrictive medications.1-3 Pharmacologic therapy is indicated for persistent or severe symptoms, with long-acting dihydropyridine calcium channel blockers as first-line treatment.1-3 Additional options for refractory cases include phosphodiesterase-5 inhibitors and topical nitrates. Raynaud phenomenon follows a variable course depending on subtype. Primary Raynaud phenomenon is generally benign, with reversible vasospasm and no tissue injury. However, a small subset of patients may progress to secondary disease over time.1 Secondary Raynaud phenomenon is associated with connective tissue disease and carries a higher risk of complications, including digital ischemia and tissue loss.1,3 Risk factors for progression include later age of onset, severe or asymmetric attacks, abnormal nailfold capillaries, and positive disease-specific autoantibodies.1,3 Anna Catinis, MD, is a dermatology resident at the Baylor College of Medicine. References - Wigley FM, Flavahan NA. Raynaud’s phenomenon. N Engl J Med. 2016;75(6):556-565. doi:10.1056/NEJMra1507638 - Rirash F, Tingey PC, Harding SE, et al. Calcium channel blockers for primary and secondary Raynaud’s phenomenon. Cochrane Database Syst Rev. 2017;12(12):CD000467. doi:10.1002/14651858.CD000467.pub2. - Block JA, Sequeira W. Raynaud’s phenomenon. Lancet. 2001;357(9273):2042-2048. doi:10.1016/S0140-6736(00)05118-7.