Idiopathic Raynaud Disease

Author:
ROBERT P. BLEREAU, MD
Morgan City, La

Citation:
Blereau RP. Idiopathic raynaud disease. Consultant. March 2013:194.

In January, a 14-year-old girl presented with purple coloration of the distal toes. During the week and a half before presentation, she had redness and swelling just proximal to the toenails. She had no history of injury to the toes. The digits were cool to touch and would hurt when pressure was applied. She had experienced anxiety and depression after her mother and father’s divorce shortly before the onset of toe discoloration.

Physical examination findings were otherwise normal. The patient had no signs or symptoms of disease, particularly of connective tissue disease, and no ulcerations.

Results of laboratory studies, including a complete blood cell count, antinuclear antibody, rheumatoid factor, anti-cyclic citrullinated peptide antibodies, and uric acid, and a chest radiograph were all normal. The patient was referred to a pediatric rheumatologist who agreed with the diagnosis of idiopathic Raynaud phenomenon.

Raynaud phenomenon, an exaggerated physiologic response to cold, with no apparent cause (idiopathic or primary Raynaud disease) often begins in adolescence and primarily manifests as asymmetrical disease without ulceration or gangrene. In contrast, secondary Ray-naud phenomenon usually develops at an older age and is associated with an underlying condition, such as a connective tissue disease, eating disorder, obstructive arterial disorder, or drug use; it also often manifests with ulcerations and gangrene. Secondary Raynaud disease occurs in 80% to 90% of persons with systemic sclerosis (it is the presenting symptom in 30%). Patients with migraine and variant angina frequently have Raynaud disease.

Distinguishing between the 2 types of Raynaud phenomenon can be challenging. Microscopy of the nail bed can help identify distorted, widened, and irregularly looped capillaries, which are characteristic of secondary Raynaud phenomenon. In primary Raynaud phenomenon, laboratory results are often normal, and patients lack a family history of the condition.¹

A hyperactive sympathetic system is thought to cause severe vasoconstriction of the distal arteries of the digits and, rarely, the earlobes, tip of the nose, and penis. The decreased blood flow can cause sensations of coldness, pain, and paresthesias. The skin changes vary from pallor to cyanosis to erythema.

The female-to-male ratio is 5:1.² Fingers are affected more than toes, which are affected in 40% of cases.² Primary Ray-naud disease is more common than secondary. It is more mild and causes fewer complications; loss of part of a digit occurs in less than 1% of patients.²

Immediate treatment for acute Ray-naud phenomenon is physical warming of the body parts. Topical application of nitroglycerin ointment may be useful in severe cases. This patient was offered nifedipine, 10 md tid, as needed for pain or burning. Other medications, such as a-blockers, angiotensin-receptor blockers, antiplatelet drugs, topical vasodilators, intravenous prostaglandins, and onabotulinumtoxin A injection, can be used depending on the cause.³ Preventive measures include avoidance of triggers (cold, vibration, and stress) and discontinuation of smoking, caffeine, stimulants, and vasoconstrictor ingestion.

This patient’s symptoms resolved in the spring when the weather became warmer.

REFERENCES:

1. Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Nelson Textbook of Pediatrics. 18th ed. Philadelphia: Saunders Elsevier; 2007:1025.
2. Fauci AS, Kasper DL, Longo DL, et al. Harrison’s Principles of Internal Medicine. 17th ed. New York: McGraw Hill; 2008:2101.
3. Bope ET, Rakel RE, Kellerman R. Conn’s Current Therapy 2012. Philadelphia: Saunders Elsevier; 2010:821.