Patient Presentation
A 4-hour-old female was seen in the newborn nursery. The infant was a full term born to a G1P1 mother with an uneventful pregnancy and delivery. She was born vaginally after a 17 hour labor. Apgars were 8 and 9 with points off for coloring, and she was examined within 30 minutes of life and noted to have a normal examination. The medical student rotating on the service presented the patient and noted that she continued to have some blue coloring of her hands and feet.

The pertinent physical exam showed an alert infant with birthweight of 3478 grams, length of 50 cm and head circumference of 34 cm; ll of which were around 50%. Her Ballard examination was consistent with gestational age of a term infant. Her examination was normal except for some very mild bluish color of her toes and fingers.

The diagnosis of a healthy infant with mild acrocyanosis was made. The attending noted that this was very common with her age, but that acrocyanosis was not that common with older children. He remarked, “Acrocyanosis can also be because of a central oxygenation problem and in this age we would worry about congenital heart disease. In older children and adults you would worry more about vasoocclusal disease. Most of the time acrocyanosis is a normal reaction to cold temperatures and goes away with time and doesn’t cause any problems. You do have to think about rheumatological problems though if you think it maybe Raynaud’s phenomenon. We’ll go over the babies’ transitioning physiology in a minute but what do you know about Raynaud’s?,” he went on to ask.

Discussion
Acrocyanosis is a peripheral vascular disease where the skin and mucous membranes have a persistent, painless, deep-bluish color due to decreased oxygenated blood which usually is benign. The deoxygenated blood can be due to a central cause such as cyanotic congenital heart disease or localized problems often due to vasospasm. Hands and feet are the most common areas affected. It is thought that there is “…vasospasm of the cutaneous arteries, and arterioles along with compensatory dilatation in the capillary and post capillary venules caus[ing] cyanosis and sweating.” Palms and soles may or may not also have hidrosis. No sensory or pulse changes are noted. Infrequently there may be some edema or other skin changes due to certain diseases. With warming or elevation of the body part, it may turn erythematous instead. Oxygen saturations are normal when tested.

In newborns acrocyanosis is pervasive as part of the normal changes from fetal to extra-uterine life. Newborns all are born with bluish extremities which then “pink up” over time as the lungs expand and the pulmonary vascular resistance decreases and the ductus arteriosus closes. This transition is not necessarily simple and may take some time to occur. Additionally, newborns also must maintain their core temperature and therefore they appropriately may not circulate as much blood to the periphery to maintain thermoregulation and therefore bluish extremities can continue to occur.

Acrocyanosis can occur because of cold exposure too, where changes to exposed areas such as body projections (e.g. ear, nose, lips) along with hands/fingers and feet/toes may be more affected. Children and younger individuals are affected more because of their smaller size. Women also appear to be more affected, again probably because of their smaller body size. The overall incidence is not known. Patients with underlying neurology or orthopaedic problems are also more affected presumably because of decreased movement (i.e. paralysis).

As most causes are benign, symptomatic treatment by avoiding cold exposure, using garments for warming, and no smoking help. Sometimes medications can also be used such as alpha-blocker or calcium-channel blocker drugs. Topical medication or surgical treatment sometimes can offer relief.

Learning Point
Differential diagnosis of acrocyanosis includes:

• Normal physiology
  • Perinatal circulatory changes
  • Cold exposure
• Raynaud’s phenomenon (RP)
  • Classic triphasic coloring is white, red and then bluish coloring
  • Usually occurs with cold exposure but individuals may have other triggering events
  • Primary RP is common and does not need treatment
  • Secondary RP causes include rheumatological problems such as systemic lupus erythematosus, mixed connective tissue disease, systemic sclerosis, Sjorgren syndrome
• Erthromelalgia
  • Uncommon
  • Name literally means red, limb, pain
  • Is a “reverse” RP where the extremities are red and painful
  • Some have a genetic component that is autosomal dominant
• Chilblain or Perniosis
  • Due to more intensive cold exposure
  • Extremities are erythematous, with burning, itching or pain
  • There is significant edema and can have tissue breakdown including blistering or worse
• Erythocyanosis
  • Location is over subcutaneous fat
  • Often associated with telangiectasis, keratosis pilaris and/or angiokeratomas
• Vasoocclusal disease
  • Pulses are low or absent in the affected area
  • Oxygen saturation is usually abnormal
  • Ulcerations are common
• Vascular skin changes
• Cutis marmorata
• Dermatomelanosis

MethhemaglobinemiaQuestions for Further Discussion
1. What are indications of possible congenital heart disease in a newborn?
A review of cyanotic congenital heart disease can be found here and A review of acyanotic congenital heart disease can be found here
2. What causes vasoocclusal disease in children?
3. What are other common normal physical examination findings are seen in the newborn period?

Related Cases

Disease: Acrocyanosis | Congenital Heart Disease

Symptom/Presentation: Cyanosis

Specialty: General Pediatrics | Neonatology

Age: Newborn

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Congential Heart Disease

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Das S, Maiti A. Acrocyanosis: an overview. Indian J Dermatol. 2013;58(6):417-420. doi:10.4103/0019-5154.119946

Erythromelalgia – NORD (National Organization for Rare Disorders). Accessed June 18, 2021. https://rarediseases.org/rare-diseases/erythromelalgia/

Goldman RD. Raynaud phenomenon in children. Can Fam Physician. 2019;65(4):264-265.

Vounotrypidis P, Pyrpasopoulou A, Sakellariou GT, et al. Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility. Clin Rheumatol. 2019;38(10):2925-2932. doi:10.1007/s10067-019-04627-w

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa