History and exam

Key diagnostic factors

common

digit pain/discomfort

Common presenting symptom.

digital paresthesia

Paresthesias can occur when the fingers are rewarming. However, paresthesias are common and nonspecific, so RP would not be considered in a patient with hand paresthesias without a history of well-demarcated pallor of fingers.[4]

pallor of digits

This must be present to diagnose primary and secondary RP.[29]

red and/or blue discoloration of digits

At least one must be present to diagnose primary RP and secondary RP.[29]

dilated capillaries at nailbeds

Occurs mainly in secondary RP; if present, secondary RP should be assumed until proven otherwise. Dilated capillaries result from dropout of capillaries with secondary hyperplasia and hypertrophy of the remaining blood vessels. On inspection, these capillaries can appear like discrete red pen marks and are often seen around the cuticle.

[Figure caption and citation for the preceding image starts]: Visible dilated capillaries at the nailbed​From the personal collection of Dr Janet Pope; used with permission [Citation ends].com.bmj.content.model.Caption@68ea8d1c

well-defined discoloration

In all cases of RP there are well-defined areas of pallor, which then display cyanosis followed by rubor.[29]

magnification of nailbeds

Presence of dilated capillaries at the nailbeds that look like red pen marks are often seen at the cuticle. This finding is highly specific for secondary RP, although not sensitive. It helps to rule in secondary RP if positive.[30]

Other diagnostic factors

uncommon

heartburn

May indicate secondary RP.

dysphagia

May indicate secondary RP.

puffy hands

May indicate secondary RP.

tight skin

May indicate secondary RP.

arthralgia

May indicate secondary RP.

photosensitivity

May indicate secondary RP.

oral/nasal ulcers

May indicate secondary RP.

alopecia

May indicate secondary RP.

butterfly rash

May indicate secondary RP.

sclerodactyly

May rarely be a complication of longstanding and frequent RP but if not in a patient with connective tissue disease, the tightening of the skin is usually distal to proximal interphalangeal joints (i.e., not entire fingers).

telangiectasia

May indicate secondary RP.

pleuritic chest pain

May indicate secondary RP.

digital ulcers

A recognized complication of RP. The presence of digital ulceration is generally thought to exclude a diagnosis of primary RP, so a secondary cause should be sought if ulceration occurs.

digital pits

A recognized complication of RP.

digital tuft resorption

A recognized complication of RP.

gangrene of fingertip/finger

A recognized complication of RP.

raised painful red lesions on finger tips

Sometimes RP features are accompanied by chilblains (perniosis), but this is a separate condition with red raised areas on tips of toes or fingers in the cold, often like frostbite. They may heal with desquamation, may be painful, are often multiple, and may be cyclical.

autoamputation

A recognized complication of RP. Nearly only in secondary RP.

Risk factors

strong

female

Primary RP is more common in women than in men.​[2][3]​​

Secondary RP is more common in women (up to 9:1) in conditions such as systemic lupus erythematosus, scleroderma, and other connective tissue diseases.

family history

In primary or secondary RP, a family history of RP or connective tissue disease may be present.​​[2][3]​​

connective tissue disease

RP occurs in 20% of patients with rheumatoid arthritis, 40% to 45% with systemic lupus erythematosus, 13% to 17% with Sjogren syndrome,​​ 10% with polymyositis, >80% with mixed connective tissue disease, and >90% with scleroderma.[13][14][15][16][17][18]​​​​​​[19]

use of certain drugs

Drug-induced RP can be caused by various drugs including beta-blockers, clonidine, ergotamine, certain chemotherapy (e.g., bleomycin, cisplatin, vincristine), cyclosporine, and stimulants (e.g., caffeine, cocaine, amphetamines). There are limited data available regarding the prevalence.[7]

vibration injury

Prevalence of RP appears to increase with exposure to vibration in the workplace.[2][20][21]​​

Vibration injury (also called hand-arm vibration syndrome) occurs from repeated use of vibrating hand-held machinery. Jackhammer operation and other causes of vibration injury increase the risk of developing RP.[22] It can cause numbness, tingling, and pain and may persist even after the occupation ceases. It occurs more often with tools of a certain acceleration amplitude and if used for longer duration. There is no diagnostic test, so the history and observed RP can be helpful.[23]

Buerger disease

Up to 40% of patients with Buerger disease (also known as thromboangiitis obliterans) experience RP.[6]​ Buerger disease involves repeated inflammation and thrombosis of digital arteries or arterioles. Smoking is a major risk factor, and chewing tobacco is also a risk. It is more common in men and often between the ages of 20 and 40 years.[24] See Buerger disease.

weak

prolonged cold exposure/frostbite

There have been case reports of RP onset after frostbite, and one population-based study reports a significant association between RP and previous frostbite.[25]​ See Frostbite.

colder climate

The prevalence of RP is higher in colder climates.[21]

smoking

Smoking can exacerbate RP.[26][27]

ischemia

Vascular ischemia (e.g., subclavian steal syndrome or peripheral vascular disease) can cause RP.

migraine

RP may be more common in people with migraines.[3]

glaucoma

RP may be more common in people with glaucoma.

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