Raynaud phenomenon in Child
Primary Raynaud phenomenon typically follows a less severe course than secondary Raynaud phenomenon. In primary Raynaud phenomenon, also termed Raynaud disease, vasospasm is not associated with ischemic injury or an underlying associated disease. Primary Raynaud phenomenon typically presents at a younger age (<30 years), involves all fingers symmetrically excluding the thumbs, and is not painful. Patients do not have a history of peripheral vascular disease or ischemic injury. Antinuclear antibody titers are normal or low (<1:40 on indirect immunofluorescence), and nail fold capillaries are normal.
Secondary Raynaud phenomenon is associated with an underlying disease, usually a connective tissue disease of which systemic sclerosis is the most common. Because vasospasm is associated with concurrent ischemia, attacks are typically painful and asymmetric involvement of digits or hands is common. Digital ulcerations or necrosis, digital tuft pits, pterygium inversum, and torturous nail fold capillaries with capillary dropout may be seen.
Additional associations include systemic lupus erythematosus, rheumatoid arthritis, pulmonary hypertension, frostbite, hematologic malignancies, polyvinyl chloride exposure, cryoglobulinemia, reflex sympathetic dystrophy, repeated trauma / vibration, arteriovenous fistula, intra-arterial drug administration, thoracic outlet syndrome, thromboangiitis obliterans, and Takayasu arteritis.
Raynaud phenomenon is considered to be rare in childhood. Raynaud phenomenon that presents in childhood should be closely worked up to exclude underlying causes. Patients should be followed closely as Raynaud phenomenon can precede other symptoms of connective tissue disease by years.
I73.00 – Raynaud's syndrome without gangrene
266261006 – Raynaud's Phenomenon
- Thoracic outlet syndrome
- Brachiocephalic trunk disease (atherosclerosis, Takayasu arteritis)
- Buerger disease (thromboangiitis obliterans)
- Crutch pressure
- Systemic sclerosis
- Systemic lupus erythematosus
- Connective tissue overlap syndromes
- Cold injury
- Vibration disease (hand-arm vibration syndrome, hypothenar hammer syndrome)
- Chemotherapy (bleomycin, vinca alkaloids, cisplatin, carboplatin)
- Vinyl chloride disease
- Arsenic poisoning
- Cryoglobulinemia (monoclonal or mixed)
- Cold agglutinin disease
- Myeloproliferative disorders
- Drug-induced Raynaud (ergot alkaloids, bromocriptine, interferon, estrogen, cyclosporine, sympathomimetic agents, clonidine, cocaine, nicotine)
- Carpal tunnel syndrome
- Carcinoid syndrome
- Reflex sympathetic dystrophy
- Pernio – itching, burning, or painful lesions on the extremities after exposure to nonfreezing cold; lesions last for 1-3 weeks
- Erythromelalgia – burning, pain, and warmth of extremities worsened by heat and improved by cold
- Cold hypersensitivity – no skin color changes
Last Updated: 05/13/2019