Photo Quiz

Discoloration in the Hands

 


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Am Fam Physician. 2015 Aug 15;92(4):295-296.

A 42-year-old woman had pain in her hands and discoloration on the tips of her fingers and toes when exposed to cold (Figure 1). She had chronic systemic lupus erythematosus that was diagnosed seven years earlier.

Her urinalysis findings were abnormal, including the presence of proteinuria and hematuria. Her creatinine level was normal, and her antinuclear antibody titer was 1:2,560. Renal biopsy, which was performed because of the proteinuria, revealed stage V membranous lupus nephritis.


Figure 1.

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Acrocyanosis.

B. Acute peripheral arterial occlusion.

C. Frostbite.

D. Raynaud phenomenon.

Discussion

The correct answer is D: Raynaud phenomenon. Raynaud phenomenon is a discoloration of the fingertips and toes after exposure to cold or emotional stress. First, the skin turns white or pale because of exaggerated vasoconstriction, then blue when the oxygen supply is depleted and red as the blood flow returns. Raynaud phenomenon may be primary or idiopathic (not associated with other conditions) or secondary (associated with other conditions). If secondary, Raynaud phenomenon can present before the diagnosis or during the natural history of the associated condition.1,2

In this patient, Raynaud phenomenon presented with a decline in kidney function. Secondary Raynaud phenomenon is often associated with autoimmune rheumatic diseases such as systemic lupus erythematosus, scleroderma, mixed connective tissue disease, Sjögren syndrome, dermatomyositis, and polymyositis. Nail fold capillaroscopy can be used to distinguish between primary and secondary Raynaud phenomenon. An abnormal result can suggest an underlying autoimmune rheumatic disease.3,4

Treatment of Raynaud phenomenon is based on lifestyle changes (e.g., keeping the hands warm, quitting smoking, exercising regularly to avoid further attacks) and on medical treatments (e.g., calcium channel blockers, topical nitroglycerin, phosphodiesterase-5 inhibitors). Local injections of onabotulinumtoxinA (Botox) or parenteral prostaglandins can also be used.2,5

Acrocyanosis is a condition characterized by a symmetric, persistent, blue or cyanotic discoloration of the hands, feet, or face. It is caused by vasospasm of the small vessels of the skin and often occurs in newborns. It is painless and considered benign.

Acute peripheral arterial occlusion because of an embolism causes an acute stoppage of blood flow. It is often associated with cardiac conditions, such as atrial fibrillation. The patient presents with acute pain, the skin turns pale and cold, and there can be paralysis of the affected area. There is no pulse to the extremity. If blood flow is not restored promptly, tissue in the area will die.

Frostbite is caused by exposure of the skin to freezing temperatures, which damages the skin and underlying tissues. Initially, the skin becomes very cold and red. If the exposure persists, the skin becomes numb, hard, and pale, then blisters and ulcers appear.

View/Print Table

Summary Table

ConditionCharacteristicsExamination findings

Acrocyanosis

Benign condition caused by mild vasospasm

Symmetric, persistent, blue or cyanotic discoloration of the hands, feet, or face

Acute peripheral arterial occlusion

Caused by an acute stoppage of blood flow as a result of an embolism

Acute pain, skin turns pale and cold, no pulse, possible paralysis of the affected area

Frostbite

Caused by freezing of the skin and underlying tissues

Skin becomes very cold and red; if the exposure persists, the skin becomes numb, hard, and pale, and blisters and ulcers appear

Raynaud phenomenon

Occurs with exposure to cold or emotional stress; caused by an exaggerated vasoconstriction

Skin turns white or pale because of vasoconstriction, then blue when the oxygen supply is depleted and red as the blood flow returns

Summary Table

ConditionCharacteristicsExamination findings

Acrocyanosis

Benign condition caused by mild vasospasm

Symmetric, persistent, blue or cyanotic discoloration of the hands, feet, or face

Acute peripheral arterial occlusion

Caused by an acute stoppage of blood flow as a result of an embolism

Acute pain, skin turns pale and cold, no pulse, possible paralysis of the affected area

Frostbite

Caused by freezing of the skin and underlying tissues

Skin becomes very cold and red; if the exposure persists, the skin becomes numb, hard, and pale, and blisters and ulcers appear

Raynaud phenomenon

Occurs with exposure to cold or emotional stress; caused by an exaggerated vasoconstriction

Skin turns white or pale because of vasoconstriction, then blue when the oxygen supply is depleted and red as the blood flow returns

Address correspondence to Antonio L. Aguilar Shea, MD, PhD, at antonio.aguilar@salud.madrid.org. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Maverakis E, Patel F, Kronenberg DG, et al. International consensus criteria for the diagnosis of Raynaud phenomenon. J Autoimmun. 2014;48–49:60–65....

2. Pope JE. Raynaud’s phenomenon (primary). BMJ Clin Evid. 2013. http://clinicalevidence.bmj.com/x/systematic-review/1119/overview.html. Accessed May 18, 2015.

3. Jung P, Trautinger F. Capillaroscopy. J Dtsch Dermatol Ges. 2013;11(8):731–736.

4. Herrick AL, Cutolo M. Clinical implications from capillaroscopic analysis in patients with Raynaud's phenomenon and systemic sclerosis. Arthritis Rheum. 2010;62(9):2595–2604.

5. Levien TL. Advances in the treatment of Raynaud's phenomenon. Vasc Health Risk Manag. 2010;6:167–177.

This series is coordinated by John E. Delzell, Jr., MD, MSPH, Assistant Medical Editor.

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