What Looks Like Acrocyanosis?

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.

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

    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

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

  • What Can You Do to Improve Compliance with Eyeglasses or Spectacle Use?

    An 18-month-old male came to clinic for his health maintenance visit.
    He had a history of congenital strabismus that had been corrected with surgery. The father noted that the eyes were much better aligned and that the amount of eye drifting that he was seeing had markedly decreased.
    However the father said that the toddler didn’t like to wear his glasses and that he wasn’t insisting on compliance as much as he had before.
    “I think he is better and he probably doesn’t need to wear them as much,” the father stated.
    The family history was positive for several children and adults with various vision problems.

    The pertinent physical exam showed a healthy appearing male without spectacles with normal vital signs and growth parameters in the 25-75%.
    The eyes were generally aligned but at the extreme lateral positions did appear not to be aligned as consistently.

    The diagnosis of strabismus was made.
    The pediatrician spoke with the father about the non-compliance with the spectacle use noting that although the patient had had improvement with surgery, the spectacle use was very important for vision now and in the future.
    The pediatrician said, “the surgery gets the eyes back into position, but the glasses keep them in position and help the eyes and brain learn how to see. It’s really really important that he use the glasses as the eye doctor told you to wear them,” the pediatrician stated.
    She went on, “I know you said that he keeps taking them off, but if you keep putting them back on then he will know that it is important and should also just get used to them. I know you have an appointment this month for followup and the eye doctor should have other ideas about how to help you keep his glasses on.”

    Uncorrected refractive errors (URE) are a major cause of visual impairment and blindness globally. It is estimated that more than 12.8 million children ages 5-15 years have URE.
    URE can cause astigmatism, anisometropia, amblyogenic hyperopia and myopia. It can also be a factor in injuries, behavioral problems and academic and social impairments.
    Spectacles (or eyeglasses or eye wear) are a low-cost, non-invasive, simple to use treatment for URE. Spectacle use has many benefits but depend on end-user use.

    Learning Point
    Non-compliance with spectacle use is estimated to be 40.1% overall with a range of 9.8-78.6% in a meta-analysis.
    Non-compliance is associated with older children, living in a rural area and in some studies females more than males.
    Compliance remains low even if the spectacles are provided at no-cost. Custom-made or ready-made spectacles do not change compliance overall.
    Compliance is improved if there is a higher RE (i.e. patient has worse vision therefore child experiences improvement using the spectacles).

    Reasons for non-compliance includes:

    • Breakage/loss/forgetfulness – 23.3%
      • Broken or lost spectacles
      • Forgetfulness
    • Personal factors – 25.8%
      • Parental and children perceptions
      • Peer pressure
      • Patient self-esteem
      • Injury concerns
      • Dislike of the spectacles, unattractiveness/poor appearance
    • Vision problems/headache – 5.5%
      • Uncomfortable spectacles
      • Unclear vision
    • Cost

    Non-vision specialists should be aware that patients with low RE may not be prescribed spectacles as they may not assist in improved vision and have a lower rate of compliance even if prescribed.
    Non-vision specialists can also discuss with patients and families their compliance and encourage adherence.

    Ways to improve compliance spectacle use includes:

    • Affordable or free spectacles can be obtained through vision insurance, governmental and charitable organizations. There are also inexpensive spectacles that can be purchased at some retail stores or on the Internet.
    • Loss and breakage – This is common and in one study where 2 pairs of spectacles were provided free to children, (1 for daily use prescribed initially and one held at school as a backup for breakage or loss), both pairs were dispensed to all children over a 1 year time period because of loss or breakage of the first pair.
      Lightweight, unbreakable and attractive frames help to mitigate many reasons for non-compliance.
    • Uncomfortable spectacles, unclear vision and headaches can be fixed by proper adjustment. Remember that adjustment may need to be performed again because of normal wearing conditions.
    • Forgetfulness can be improved with adult encouragement including parents and school personnel. Also having access to a second pair of spectacles at school or home also improves compliance.
    • Social barriers – parents should be educated about the importance of spectacle use for current and long-term vision outcomes, so they can encourage compliance. Children can be taught ways to mitigate teasing and bullying over their appearance.
      Acknowledging that there may be societal factors (i.e. child is seen as have a disability or is less desirable as a potential marriage partner) which may discourage use but individual use is important for the overall lifetime health of the patient.

    Questions for Further Discussion
    1. What are some of your own recommendations for increasing compliance with spectacle use?
    2. How good is photoscreening for vision? A review can be found here
    3. At what refractive error on visual screening should a child be referred to a vision professional?
    4. What are indications for eye patching?

    Related Cases

    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 these topics: Eye Wear and Vision Impairment and Blindness.

    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.

    Maconachie GDE, Farooq S, Bush G, Kempton J, Proudlock FA, Gottlob I. Association Between Adherence to Glasses Wearing During Amblyopia Treatment and Improvement in Visual Acuity. JAMA Ophthalmol. 2016;134(12):1347-1353. doi:10.1001/jamaophthalmol.2016.3793

    Morjaria P, McCormick I, Gilbert C. Compliance and Predictors of Spectacle Wear in Schoolchildren and Reasons for Non-Wear: A Review of the Literature. Ophthalmic Epidemiol. 2019;26(6):367-377. doi:10.1080/09286586.2019.1628282

    Dhirar N, Dudeja S, Duggal M, et al. Compliance to spectacle use in children with refractive errors- a systematic review and meta-analysis. BMC Ophthalmol. 2020;20(1):71. doi:10.1186/s12886-020-01345-9

    Hark LA, Thau A, Nutaitis A, et al. Impact of eyeglasses on academic performance in primary school children. Can J Ophthalmol. 2020;55(1):52-57. doi:10.1016/j.jcjo.2019.07.011

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

    What Are the Advantages and Disadvantages of Different Renal Replacement Therapies?

    Patient Presentation
    A 6-month-old male came to clinic for his health maintenance visit. He had an eventful past medical history including being diagnosed with posterior urethral valves in-utero with ablation and vesicostomy after birth. His kidney function was currently stable but the family was anticipating his kidney function to progress to end stage kidney disease as there already had been some decline. Although he was growing, he had poor weight gain and linear growth and also had had 1 urinary tract infection. He seemed to be developing appropriately and was saying consonants, sitting up and interacting with the family appropriately. The family history was negative for genetic or renal disease.

    The pertinent physical exam showed a thin male growing at the 3rd percentile for weight and 5% for length. Blood pressure was borderline at 102/64. He had the vesicostomy in place along with an NG tube that provided overnight supplemental nutrition. His examination was otherwise unremarkable.

    The diagnosis of a patient status-post posterior urethral valve surgery with chronic renal disease was confirmed. The pediatrician offered support to the parents and discussed what he could do to help the family. “You need to be working with the kidney doctors and surgeons mainly, but I can help if he gets sick or you aren’t sure who to call. You can always call me and my partners,” he offered. “I am also willing to be there when some of the big decisions are going to have to be made such as when to do a transplant or start dialysis or something else. I’m not a kidney specialist but I know about kids and your family so I can be there to help you if you want,” he said.

    Kidneys are amazing in that they filter the blood, eliminate toxins, balance fluids and electrolytes plus have a lot of reserve to do the job they need to do even when injured. Therefore, depending on the problem, chronic renal disease (CRD) often allows time before renal replacement therapy (RRT) is needed. RRT currently is dialysis (peritoneal or hemodialysis) or renal transplant. In general, the patient’s own kidneys do the best job for them but there is always a balance between the problems of CRD and its management, versus the potentially improved renal function with RRT. RRT itself though has a different set of management problems.

    Causes of end stage renal disease include:

    • *Congenital
      • Hypoplasia/dysplasia (10%)
      • Obstructive uropathies (9.5%)
    • *Cystic
      • Polycystic kidney disease
    • *Hereditary
    • Primary glomerular disease (25%) – mainly focal segmental glomerulosclerosis
    • Secondary glomerulonephritis and vasculitis (12%) – mainly lupus nephritis

    Congenital, cystic and hereditary combined are the most common grouping which accounts for about 38%.

    Primary care providers can support patients and families in their treatment and management decisions, they can promptly evaluate and coordinate care with specialists for acute problems, and provide immunization for all non-live virus vaccines to patients and make sure that family members have all of their own vaccines including live-virus vaccines. Mental health support at all times but especially around anticipated life transitions can help families too. Counseling about long-term health behaviors is important to try to decrease the risk of malignancy and cardiovascular problems including scrupulous use of sun protection measures, no tobacco use, little or no alcohol use, weight management and nutrition, and avoiding dehydration.

    Learning Point
    ESRD itself carries some innate problems including anemia and mineral bone disorders. Life expectancy varies greatly depending on the underlying disease, management and treatment. Overall, life expectancy is reduced for both genders

    RRT options and their advantages and disadvantages includes:

    The number of children on dialysis who have other co-morbidities is increasing and therefore these patients may be more complex. Both types of dialysis can have problems with the catheter or infections. For children, peritoneal dialysis is usually preferred over hemodialysis.

    Peritoneal dialysis is used as the first dialysis modality for 80-90% of patients. It doesn’t need vascular access, there are fewer dietary restrictions and better catchup growth. It can also be done at home usually overnight, which may be more helpful to the overall family functioning. But this also means that much of the care is reliant on the family to do the dialysis, give multiple medications, monitor feedings, blood pressure, etc.

    Hemodialysis has the advantage that much of the care is done by others but there still is the problem of obtaining and maintaining vascular access, increased risk of hemodynamic instability, risk of infection, amount of time to travel and be at a dialysis center. There is also an increased risk of anemia.

    Kidney transplant


    • For children kidney transplant offers many advantages and currently is the preferred form of RRT if possible
    • For many patients, shorter times for dialysis and preemptive transplant (patient has kidney transplant without dialysis) may provide longer graft survival
    • With transplant, patients have a 10 year survival of 90-95%
    • Graft survival is about 12-15 years in children. Therefore children with ESRD often require more than one transplant over their lifetime
    • Living related donor transplants have better long-term outcomes than non-living related donors


    • Size – newborns and infants may not be large enough to receive a transplant. Patients need to be around 8-10 kg in size generally.
    • Graft – acute or chronic grade rejection, progression of the CRD or recurrence of the primary kidney disease
      Rejection can be difficult to diagnose particularly as there is renal reserve especially in young children who have a large graft relative to their muscle mass, and therefore creatinine levels may have no change. Acute rejection can also be asymptomatic or present with mild fever or mild hypertension.

    • Immunosuppression/Infection – patient is immunocompromised, primary viral diseases may cause severe disease such as cytomegalovirus, Epstein-Barr virus and BK polyoma virus
      Diarrhea can cause serious problems as even mild dehydration can significantly alter the kidney function and absorption and metabolism of the immunosuppressant medications.
      UTI is also common.

    • Malignancy is very common in adult survivors who had pediatric kidney transplant. ~40% in one study with most being nonmelanoma skin cancer but also a variety of other malignancies
    • Cardiometabolic problems – hypertension, diabetes, hyperlipidemia, obesity, for children and adults hypertension, cardiovascular and valve disease are the main long-term causes of mortality
    • Growth/development – short stature, developmental delay, delayed puberty, rickets, psychosocial problems related day-to-day activities but also during transition times such as puberty, emerging adulthood and fertility.

    Questions for Further Discussion
    1. How is renal transplant similar and different to other transplants?
    2. What factors influence growth after renal transplant? A review can be found here
    3. What causes proteinuria? A review can be found here

    Related Cases

    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 these topics: Kidney Failure, Chronic Kidney Disease Kidney Transplant, and Dialysis.

    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.

    Neild GH. Life expectancy with chronic kidney disease: an educational review. Pediatr Nephrol. 2017;32(2):243-248. doi:10.1007/s00467-016-3383-8

    Winterberg PD, Garro R. Long-Term Outcomes of Kidney Transplantation in Children. Pediatr Clin North Am. 2019;66(1):269-280. doi:10.1016/j.pcl.2018.09.008

    Sanderson KR, Warady BA. End-stage kidney disease in infancy: an educational review. Pediatr Nephrol. 2020;35(2):229-240. doi:10.1007/s00467-018-4151-8

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

    How does Lichen Sclerosus et Atrophicus Present?

    Patient Presentation
    A 9-year-old female came to clinic with a 1 week history of perineal irritation and dysuria. This occurred mainly during the day, but it had awoken her from sleep. Her mother described the area as reddened and was not improving with sitz baths and emollients. She described that she didn’t have to urinate more frequently, nor did starting the urine stream bother her, but once the urine started flowing she had a pain. She had been afebrile and was otherwise feeling well. She denies any new soaps, detergents or other products including no new clothing. She wore cotton underwear and loose fitting clothing generally. She denied vaginal discharge and said that no one had touched her inappropriately in her genital area. She had no previous history of urinary tract infections and denied anal itching or pain. She did not have any significant dermatological or allergic history. There was no travel or animal contacts. The family history was positive for diabetes and maternal family members with thyroid problems.

    The pertinent physical exam showed a well-appearing female with normal vital signs and growth parameters in the 10-50%. Her general skin examination showed no lesions. Her genital area revealed a pre-pubertal female with a pale white, shiny, continuous lesion involving the labia majora bilaterally that extended to the perineum. It did not extend to the anus and there was reddened skin lateral to the white area. It was well-demarcated but not “punched out” appearing. The affected area seemed thin and there were excoriations. There was no excessive vaginal discharge or odor. The hymen and other genital structures appeared normal.

    The differential diagnosis of a urinary tract infection, post-irritation hypopigmentation such as due to pinworms, vitiligo or lichen sclerosus et atrophicus was considered. Vaginal foreign body or vulvovaginitis were considered but less likely given the physical examination. The attending pediatrician remarked to the resident, “This is a good example of why we rotate through all the specialties in medical school. We need to think about other things, but I think this is LS&A. I’ve seen it a couple of times in practice, but I learned about it in the adult gynecology clinic.” The resident pediatrician agreed that the patient’s lesion did appear similar to her experiences with adult women as well. “This isn’t the classic appearance but looks more like the top half of the hourglass,” the resident said. The laboratory evaluation of a urinalysis was normal. The patient was referred to the gynecology clinic who saw her the following day and confirmed the diagnosis. She was started on topical corticosteroids.

    Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disease with a strong autoimmune association. It can occur with other autoimmune diseases. Its specific cause is unknown. It affects females and males of all ages, but especially females 40-60 years. In the pediatric population it affects prepubertal females most often.

    Treatment includes steroid medications or anti-inflammatory medications. Other treatments may be offered including phototherapy, and much less commonly systemic steroids or immunosuppressive therapy. Circumcision may also be an option for some males. Childhood LSA generally improves but can continue into adulthood. Scarring can occur with narrowing of the openings of the urethra, vagina, and anus and also the foreskin in males. Malignancy has been reported in adults but not children.

    The differential diagnosis includes post-inflammatory hypopigmentation, lichen planus, tinea versicolor, vitiligo or morphea (localized sclerosis). Malignancies should also be considered in older populations. Vulvovaginitis and sexual abuse should be considered in the differential diagnosis for children because of the genital irritation presentation but usually do not have the same physical examination appearance.

    Learning Point
    LSA lesions are shiny, and pale or white. The lesion edges can appear more diffuse or well-demarcated. The tissue appears shiny, thin, wrinkled and parchment-like, while others will describe it as a “porcelain white appearing center surrounded by redness.”

    The classic presentation of LSA are genital lesions that are described as hour-glass or a figure of 8 for females as it involves the vulva, perineum and anal areas. For males the most common involvement is of the foreskin which presents as phimosis. The glans and meatal involvement are also relatively common in males. Genital involvement tends to be diagnosed more quickly as it is commonly quite symptomatic with burning or pruritis. There can also be blistering or fissuring of the area. Constipation or dysuria due to pain from the fissuring area are common presentations as well. Extra genital involvement includes lateral thighs, buttocks, axilla and upper trunk including the breast. But can include other areas such as the face or orally. Extra-genital lesions tend to be diagnosed later as they are less symptomatic.

    Questions for Further Discussion
    1. What is in the differential diagnosis for vulvovaginitis? A review can be found here
    2. What is the current antibiotic of choice for uncomplicated cystitis?
    3. What are indications for referral to a gynecologist or urologist?
    4. What are relative indications for medical circumcision?

    Related Cases

    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 these topics: Vulvar Disorders and Skin Conditions.

    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.

    Pope E, Laxer RM. Diagnosis and management of morphea and lichen sclerosus and atrophicus in children. Pediatr Clin North Am. 2014;61(2):309-319. doi:10.1016/j.pcl.2013.11.006

    Tomo S, Santos I-S, de Queiroz S-A, Bernabe D-G, Simonato L-E, Miyahara G-I. Uncommon oral manifestation of lichen sclerosus: critical analysis of cases reported from 1957 to 2016. Med Oral Patol Oral Cir Bucal. 2017;22(4):e410-e416. doi:10.4317/medoral.21606

    Akel R, Fuller C. Updates in lichen sclerosis: British Association of Dermatologists guidelines for the management of lichen sclerosus 2018. Br J Dermatol. 2018;178(4):823-824. doi:10.1111/bjd.16445

    Green PA, Bethell GS, Wilkinson DJ, Kenny SE, Corbett HJ. Surgical management of genitourinary lichen sclerosus et atrophicus in boys in England: A 10-year review of practices and outcomes. J Pediatr Urol. 2019;15(1):45.e1-45.e5. doi:10.1016/j.jpurol.2018.02.027

    Lichen Sclerosus. NORD (National Organization for Rare Disorders). Accessed May 18, 2021. https://rarediseases.org/rare-diseases/lichen-sclerosus/

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