Chilblains: An Old But Now More Common Problem

Patient Presentation
A 13-year-old female came to clinic with a 2-day history of foot discoloration, pain, pruritis and edema. Walking or pressure did not change the symptoms, but exposure to a heating pad or warm water soaks did improve them. She denied any problems elsewhere in her body, and was not known to have had frostbite, previous cold exposures or reactions to cold such as a Raynaud-like reaction. She denied any medications and had no fever, weight change, cold or heat intolerance, eye changes, or joint or muscle problems.

The past medical history showed she had tested positive for COVID-19 along with her family approximately 3 weeks previously. During that time she had mild cough and fever that resolved within a few days without additional problems. The family history was negative for autoimmune or rheumatological diseases but had some heart attacks and strokes.

The pertinent physical exam showed a healthy female with normal vital signs. Her growth was at the 50-75%. Her examination was normal except for her feet which were mottled or a reddish-purple to whitish coloring. Most of the foot was involved including the heel but changes stopped around the malleoli. Her middle toes bilaterally were somewhat swollen and she said had intermittent pain or itching.

The diagnosis of chilblains was made. It was thought to be due to her recent COVID-19 infection. She was started on topical steroids and recommended to wear warm socks and monitor for additional symptoms. She and her guardian were counseled regarding chilblains nature history also.

Discussion
Chilblains is also known as perniosis is an “…inflammatory dermatosis that generally affects the dorsal feet or hands during the periods of damp and cold, but not freezing, conditions…” It produces acryl lesions.

Chilblains may be derived from the Old English words “chill” and “blegen” (sore). Pernio is thought be derivative of pern(a) or haunch of the leg as the feet and legs are commonly affected. Chilblain is usually idiopathic but has been associated with Raynaud disease, blood problems including cryofibrinogenemia and rheumatologic conditions such as systemic lupus erythematosus, rheumatoid arthritis and antiphospholipid syndrome.

It should be distinguished from livedo reticularis and usually is more easily distinguished from acrocyanosis. Livedo reticularis is a net-like, red-purple skin discoloration that is caused by abnormal circulation in the affected area. It can simply occur because the area or the person is cold. Acrocyanosis a persistent, painless, deep-bluish color due to decreased oxygenated blood which usually is benign. A review and differential diagnosis of acrocyanosis can be found here.

Since the COVID-19 pandemic, the diagnosis of chilblains has increased along with other vascular and dermatological conditions such as MIS-C.
Data is early but increasingly there is a strong association with COVID-19.

Learning Point
Chilblains due to COVID appears the same histologically as other etiologies.

A retrospective cohort study with geolocation of potential cases and COVID-19 incidence found that there was a large increase in case incidence during the COVID-19 pandemic but there was low testing positivity and therefore a weak correlation with COVID-19 incidence. Before COVID-19 the annual incidence (2016-2020) was 5.2 per 100,000 person-years, but rose to 28.6 during the pandemic. The authors hypothesize that this may be due to more children being affected and who may not be symptomatic of COVID, or may not mount as robust an immune response so is a false-negative testing, or the testing itself could be a problem.

Before the pandemic, 20-39 year olds and > 60 year old had the highest incidence. During the pandemic, school age children had the highest incidence rising from before the pandemic from 9.1 to 62.7 per 100,000 person years. This is approximately 3x the incidence of the other age groups studied. Before and during the pandemic Asian-American and White races were more affected than others (at a much higher rate for all groups) and females were more affected than males.

Another study of pooled case reports and observational studies (N = 715) found that the average patient age was 16.6 years old, and had a slight male prevalence. Chilblains occurred in the feet in 91.4%, hands in 17.9% and both in 11.7%. Foot involvement was even more common in those under 20 years than those over 20 years old (92% vs 72.6%). Pruritis (35.8%) and pain (24.8%) were common but 35.7% were asymptomatic. Common presentation was macules and papules that were red or violaceous with edema occurring in 31.8% for those under 20 years. Potential COVID-19 infection was reported in 50.2%.

In studies, overall patients did well with most having improvement in symptoms within a few weeks (up to 60 days has been reported). Some patients were treated with topical steroids which may improve symptoms.

Questions for Further Discussion
1. How are MIS-C and Kawasaki Disease different than chilblains? A review can be found here.
2. How do you treat immersion foot injury? A review can be found here.
3. How do steroid medication supposedly improve symptoms of chilblains?
4. How do you grade frostbite? 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: Frostbite and Raynaud’s 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.

Definition of pernio. Dictionary.com. http://www.dictionary.com. Accessed February 22, 2022. https://www.dictionary.com/browse/pernio

Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus–a review of literature. Clin Rheumatol. 2008;27(8):949-954. doi:10.1007/s10067-008-0942-9

Kolivras A, Thompson C, Pastushenko I, et al. A clinicopathological description of COVID-19-induced chilblains (COVID-toes) correlated with a published literature review. J Cutan Pathol. Published online August 9, 2021:10.1111/cup.14099. doi:10.1111/cup.14099

Rocha KO, Zanuncio VV, de Freitas BAC, Lima LM. “COVID toes””: A meta-analysis of case and observational studies on clinical, histopathological, and laboratory findings. Pediatr Dermatol. 2021;38(5):1143-1149. doi:10.1111/pde.14805

Epidemiologic Analysis of Chilblains Cohorts Before and During the COVID-19 Pandemic | Dermatology | JAMA Dermatology | JAMA Network. Accessed February 22, 2022. https://jamanetwork.com/journals/jamadermatology/fullarticle/2781362

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

Are These Stools Acholic?

Patient Presentation
A 3-year-old male came to clinic with a history of pale stools off and on for 1 week. He was toilet training and so his mother was very aware of his elimination patterns. He had daily bowel movements which she said they were “pale” for a couple of days, then brown, then “pale” again. The color was uniform within soft, toothpaste consistency stools. She denied seeing any foreign material within the stool. He was otherwise acting normally and appeared well and was eating normally, and had clear “light-colored” urine multiple times a day. He had no rashes, fevers, chills, emesis or pain. She didn’t believe there were any weight changes. She denied any medications. The past medical and family history were non-contributory.

The pertinent physical exam showed a well-appearing male with growth parameters in the 50% range and was tracking from a visit 1 month previous. HEENT showed no scleral icterus. His skin had no jaundice or abnormal vein patterns. His abdomen was soft with normal size liver and spleen. No masses were palpated. He had some shotty anterior cervical and inguinal nodes. His examination was otherwise unremarkable.

The diagnosis of potentially abnormal colored stools was made.
Pictures provided by the mother within the toilet showed what appeared to be pale yellow stools that were uniform in color. The child otherwise appeared well so the physician told the mother that these appeared normal but to continue monitoring the patient for white or grey stools, or any signs that the patient was unwell.

Discussion
Color can be difficult to discern and communicate. Stooling is an important part of overall health and an important harbinger of potential illness. Stooling consistency and color changes with food, environment, medication, and health conditions.

For neonates and young infants, some hepatobiliary problems may not be seen at birth but in the next few days to weeks. This includes Alagille syndrome, biliary atresia and cystic fibrosis. It is not uncommon for these patients to have prolonged jaundice and therefore the diagnosis may be delayed with some presenting with increased jaundice, acholic stool and poor feeding. In older children causes of white/pale/clay-colored/grey stool may be easier to determine. Patients with underlying health problems usually have a different history (i.e. potential infectious hepatitis or medication exposure, increasing scleral icterus, poor eating, fever, etc.) and usually different physical examination. For a well-appearing child without concerning history or physical examination findings, the most common cause is a misperception or reporting of the change.

The differential diagnosis of acholic stools includes:

  • Biliary disease
    • Liver
      • Alagille syndrome
      • Biliary atresia
      • Cystic fibrosis
      • Cirrhosis, end-stage liver disease
      • Infectious hepatitis
      • Drug-induced hepatitis
      • Tumors
    • Gallbladder
      • Anatomical problems
      • Biliary stricture
      • Gallstones
      • Sclerosing cholangitis
      • Tumors
    • Pancreas
      • Pancreatitis
      • Tumor
  • Foreign bodies – crayons, paint, toys, worms
  • Malabsorption and undigested food
  • Medications – barium, antacids
  • Misperception of color
  • Mucus
  • Tumors, abdominal

A review of different color stools can be found here.

Learning Point
True acholic stools should be appropriately evaluated usually with a gastroenterologist. As colors are difficult to discern, neonatal stool color cards have been used to help parents. There is also a mobile phone application which can help with classification of stool color. Images of neonatal stool color cards and acholic stools can be reviewed in To Learn More below.

Questions for Further Discussion
1. What is the most common reason for “pale or white” stool in your practice?
2. How do you approach the concern of “blood in stool?”
3. What would you include in your evaluation for acholic stools?

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: Bowel Movement and Digestive Diseases.

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.

Chen SM, Chang MH, Du JC, et al. Screening for biliary atresia by infant stool color card in Taiwan. Pediatrics. 2006;117(4):1147-1154. doi:10.1542/peds.2005-1267

Gu YH, Yokoyama K, Mizuta K, et al. Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan. J Pediatr. 2015;166(4):897-902.e1. doi:10.1016/j.jpeds.2014.12.063

Shneider BL, Moore J, Kerkar N, et al. Initial assessment of the infant with neonatal cholestasis – Is this biliary atresia? PLoS One. 2017;12(5):e0176275. doi:10.1371/journal.pone.0176275

Madadi-Sanjani O, Blaser J, Voigt G, Kuebler JF, Petersen C. Home-based color card screening for biliary atresia: the first steps for implementation of a nationwide newborn screening in Germany. Pediatr Surg Int. 2019;35(11):1217-1222. doi:10.1007/s00383-019-04526-w

Singh H, Hong MH, Hinds R. Acholic stools and a small gallbladder: Not always a case of biliary atresia. Journal of Paediatrics and Child Health. 2020;56(11):1812-1813. doi:10.1111/jpc.14694

Angelico R, Liccardo D, Paoletti M, et al. A novel mobile phone application for infant stool color recognition: An easy and effective tool to identify acholic stools in newborns. J Med Screen. 2021;28(3):230-237. doi:10.1177/0969141320974413

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

What is in the Differential Diagnosis for Brown and Grey Skin Conditions?

Patient Presentation
A 14 year-old male came to clinic for his health examination visit and to followup for obesity. He had increased his physical activity over the past 6 months but had not made dietary changes.

The pertinent physical exam showed an obese male with normal vital signs. He had gained 3.4 kg since his last visit and had increased his BMI to 37.2. His skin showed velvety-brown lesions on his neck and upper back. The rest of his examination was non-contributory. The diagnosis of a teenage male with obesity, acanthosis nigricans and increasing BMI trend was made. He was commended for increasing his exercise but also counseled again about healthy eating. After his laboratory evaluation showed abnormal lipids, hemoglobin A1c of 6.7%, and elevated liver enzymes, he was also referred to the cardio-metabolic clinic for additional evaluation and management including meeting with a dietician.

Discussion
This is the fourth in a short case series of differential diagnoses of colored skin conditions.
An introduction to dermatological terminologies and information about colors can be found here.
A differential diagnosis by distribution and common pattern can be found here.
For red, orange and yellow conditions, a review can be found here.
For green, blue and purple conditions, a review can be found here.
For black and white conditions, a review can be found here.

Note that any color can be a normal variant for an individual or is physiologic for a given state.

Acanthosis nigricans causes dark brown-black patches or streaks in skin creases especially on the neck, armpits and groin. It has a velvety elevated texture. It is caused by insulin resistance.

Learning Point
Brown is produced in several ways. Combinations of red, yellow and black, or orange and black are the usual ways.
The differential diagnosis for brown skin conditions includes:

  • Skin
    • Acanthosis nigricans
    • Addison disease
    • Cushing syndrome
    • Drug induced hyperpigmentation
      • Topical or systemic medications often with additional skin exposure
    • Incontinentia pigmenti
    • Neoplasms
      • Langerhans cell histiocytosis
      • Lymphoma
      • Melanoma
    • Pigmented purpuric dermatosis
      • Petechiae
      • Purpura
      • Telangiectasis
    • Post-inflammatory hyperpigmentation
      • Cosmetics
      • Inflammation
      • Sun
      • Trauma
    • Pregnancy
    • Melasma
    • Riehl melanosis
    • Seborrheic keratosis
    • Stasis dermatitis
  • Nails
    • Chemical-induced
    • Incontinentia pigmenti
    • Melanonychia
    • Trauma
  • Mucosa
    • Infection
    • Peutz-Jegher syndrome
    • Laugier-Hunziker syndrome
    • Trauma

Grey is a combination of white and black. “An achromatic gray is a gray color in which the red, green, and blue codes are exactly equal….A chromatic gray is a gray color in which the red, green, and blue codes are not exactly equal, but are close to each other, which is what makes it a shade of gray.”
The differential diagnosis for gray skin conditions includes:

  • Skin
    • Ashy dermatitis (erythema dischromicum)
      • Dermal melanocytosis
      • Congenital dermal melanocytosis
      • Nevus of Ito or Ota
    • Drugs often with additional skin exposure
      • Antibiotics
        • Grey-baby syndrome with chloramphenicol
        • Tetracycline
      • Heavy metals
        • Gold
        • Iron
        • Silver
    • Incontinentia pigmenti
    • Hypomelanosis of Ito
    • Hemochromatosis
    • Lichen planus pigmentosis
    • Naegeli-Franceschetti-Jadassohn syndrome
    • Ochronosis
    • Organ failure (late)
    • Trauma
  • Hair
    • Chemical-induced
    • Silvery hair syndrome
  • Nails
    • Chemical-induced
    • Incontinentia pigmenti

Questions for Further Discussion
1. What brown skin conditions do you see most often?
2. What grey skin conditions do you see most often?
3. What are indications for referral to a dermatologist?

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: Rashes

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.

Abdel-Naser MB. The color of skin: gray diseases of the skin, nails, and mucosa. Clinics in Dermatology. 2019;37(5):507-515. doi:10.1016/j.clindermatol.2019.07.011

Kutlubay Z, Cesur SK, Askın O, Tuzun Y. The color of skin: brown diseases of the skin, nails, and mucosa. Clinics in Dermatology. 2019;37(5):487-506. doi:10.1016/j.clindermatol.2019.07.007

Shades of brown. In: Wikipedia. 2022. Accessed January 25, 2022. https://en.wikipedia.org/w/index.php?title=Shades_of_brown&oldid=1065742420

Shades of gray. In: Wikipedia. 2022. Accessed January 25, 2022. https://en.wikipedia.org/w/index.php?title=Shades_of_gray&oldid=1065573955

Visible spectrum. In: Wikipedia. 2021. Accessed January 4, 2022. https://en.wikipedia.org/w/index.php?title=Visible_spectrum&oldid=1062416030

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

What is in the Differential Diagnosis for Black and White Skin Conditions?

Patient Presentation
A 2 year-old African-American female came to clinic with white spots her mother was concerned because they were worsening. The spots were mainly on her face and arms but also a few on her trunk and had been worsening in that they were more prominant for about 1 month. The mother denied any new soaps, lotions/detergents or other products. She was using sunscreen intermittently. She had been to the pool more often because it was summer. She was otherwise well. The past medical story was positive for atopic dermatitis.

The pertinent physical exam showed a healthy appearing toddler with normal vital sign and growth parameters in the 50%. She had mild, generalized xerosis but also areas where she had more patches of reddened skin. She also had similar areas of 1-3 cm in size that were lighter to quite white in color with a blended border that was not punched out or discrete. The red and white patches were mainly on the face, upper trunk and extremities.

The diagnosis of atopic dermaitis with post-inflammatory skin changes was made. The physician explained that most likely the combination of atopic dermatitis, pool and possibly sunscreen chemical exposure probably was irritating her skin, and after the inflammation there can also be either lightening or darkening of the skin.
The sun exposure could also make her skin darken through tanning, all of which makes the white areas more prominent. She recommended continuing to use the sunscreen as the toddler had not had reactions to it in the past. She also recommended that the child shower after pool exposure and put on liberal amounts of additional sunscreen or emollients. Topical steroids were not recommended as she thought that emollients would help with the reddened areas. “If we can stay away from steroids I always try to do that. Plus steroids can also cause lightening of the skin as well for some people,” she explained. She also reviewed pool and summer safety with the mother.

Discussion
This is the third in a short case series of differential diagnoses of colored skin conditions.
An introduction to dermatological terminologies and information about colors can be can be found here.
A differential diagnosis by distribution and common pattern can be found here.
For red, orange and yellow conditions, a review can be found here.
For green, blue and purple conditions, a review can be found here.
For brown and grey conditions, a review can be found here.

Note that any color can be a normal variant for an individual or is physiologic for a given state. When lesions of the opposite color of the normal skin tone occur, this can be worrisome for individuals.

From a physics standpoint, black and white are not colors. Black and white do not have specific visual spectral wavelengths. Instead, black absorbs all the waves in the visible spectrum (none are reflected back to see the color), and white reflects back all the wavelengths. From an artistic or daily living viewpoint, black and white are described/treated as colors.

Learning Point
Black is not in the visible spectrum of light.
The differential diagnosis for black skin conditions includes:

  • Skin
    • Acne – blackhead or open comedome
    • Acanthosis nigricans
    • Blue nevus
    • Calciphylaxis
    • Dermatosis papulose nigra
    • Discoid lupus erythematosus
    • Ecthyma gangrenosum
    • Exogenous ochronosis
    • Foreign body
    • Hematoma
    • Ink spot lentigo
    • Infection
      • Mucormycosis
      • Tinea nigra
      • Verrucae plantaris
    • Neoplasms
      • Basil cell carcinoma
      • Melanoma
      • Reed nevus or pigmented spindle nevus
      • Seborrheic keratosis, pigmented
    • Staining
      • Black dermatographism
      • Tattoo – intentional or traumatic
    • Vasculitis and embolism
      • Black heel or calcaneal petechiae
      • Frostbite
      • Gangrene
      • Thromboembolic disease
  • Hair
    • Black dot tinea capitus
    • Black piedra
  • Nail
    • Subungual hematoma
    • Melanonychia
  • Mucosa
    • Amalgam reactions
    • Black harry tongue

White is not in the visible spectrum of light
The differential diagnosis for white skin conditions includes:

  • Skin
    • Actinic keratosis
    • Albinism
    • Blanching, normal
    • Degos disease
    • Chemical exposure including lead
    • Halo nevus of Sutton
    • Happle syndrome
    • Hand-arm vibration syndrome
    • Idiopathic guttate hypomelanosis
    • Intralesional steroid injection
    • Lichen sclerosus et atrophicus
    • Lichen striatus
    • Melasma
    • Milia
    • Localized hypopigmentation including ash-leaf spots
    • Piebaldism
    • Pityriasis alba
    • Pityriasis licenoides chronica
    • Psoriasis
    • Post-inflammatory reaction
      • Atopic dermatitis
      • Candidal dermatitis
    • Sun bleaching
    • Tinea versicolor
    • Urticaria
    • Vitiligo
    • Waardenburg syndrome
  • Hair
    • Chemical exposure
    • Depigmentation
    • Sun bleaching
    • UV light leukoderma
  • Nail
    • Leukonychia
    • Trauma
  • Mucosa – often temporary
    • Trauma
    • Infection

Questions for Further Discussion
1. What black skin conditions do you see often?
2. What white skin conditions do you see often?
3. What are indications for referral to a dermatologist?

Related Cases

    Symptom/Presentation: Rash

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: Rashes

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.

Brown AE, Qiu CC, Drozd B, Sklover LR, Vickers CM, Hsu S. The color of skin: white diseases of the skin, nails, and mucosa. Clinics in Dermatology. 2019;37(5):561-579. doi:10.1016/j.clindermatol.2019.07.018

Qiu CC, Brown AE, Lobitz GR, Shanker A, Hsu S. The color of skin: black diseases of the skin, nails, and mucosa. Clinics in Dermatology. 2019;37(5):447-467. doi:10.1016/j.clindermatol.2019.08.003

Visible spectrum. In: Wikipedia. 2021. Accessed January 4, 2022. https://en.wikipedia.org/w/index.php?title=Visible_spectrum&oldid=1062416030

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