Case history
Case history #1
An 18-year-old healthy woman presents with fingertips that turn white, then blue and red, in the cold. She is otherwise well and is a non-smoker. Her symptoms have been occurring for 3 years. Her mother has RP. The remainder of her history and physical examination is unremarkable. She has a normal full blood count and a negative antinuclear antibody. She has primary RP.
Case history #2
A 50-year-old woman has developed puffy hands, new RP, and difficulties swallowing over the last year. Her chest and abdominal examinations are unremarkable. She does not have inflammatory arthritis. She has some small dot-like telangiectasia on her hands and lips. Her antinuclear antibody is positive and has a nucleolar pattern. There are dilated capillaries seen at the nailbed, proximal to the nail, on several fingers. She currently has a digital ulcer. She has systemic sclerosis (scleroderma).
Other presentations
RP causes pain, discomfort, and sometimes paraesthesia. Rarely, ulcers on the fingers and toes occur (and much less commonly on the ears, nose, hand, wrist, or forearm). More severe cases usually occur only when RP is secondary to an underlying cause, in particular connective tissue disorders such as systemic sclerosis (SSc), scleroderma, systemic lupus erythematosus, Sjogren's syndrome, rheumatoid arthritis, or polymyositis. Secondary RP is associated with vasculitis, severe peripheral vascular disease (such as Buerger's disease), and rarely malignancy or chemotherapy. Environmental trauma (e.g., excessive vibration exposure in jackhammer operators, frostbite) can precipitate RP. In medium-vessel vasculitis disorders such as polyarteritis nodosa and occasionally systemic lupus erythematosus, gangrene of the fingertip or entire finger may occur.
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