What are the other Names for this Condition? (Also known as/Synonyms)

• Erythrokeratolysis Hiemalis
• KWE (Keratolytic Winter Erythema)
• Oudtshoorn Disease

What is Keratolytic Winter Erythema? (Definition/Background Information)

• Keratolytic Winter Erythema (KWE) is an inherited (autosomal dominant) disorder that is characterized by red discoloration and peeling of skin on the palms and soles, with onset and exacerbation during winters
• The condition is predominantly reported among Afrikaners from the nation of South Africa. It was initially described among certain families in the town of Oudtshoorn in Western Cape, South Africa. Hence, KWE is also known as Oudtshoorn Disease
• Presently, there are no well-defined treatments available for Keratolytic Winter Erythema. The condition may get better as the individual grows older. The prognosis of KWE depends on its severity; individuals with mild conditions have better outcomes than those with severe skin disease

Who gets Keratolytic Winter Erythema? (Age and Sex Distribution)

• Keratolytic Winter Erythema is observed during infancy, childhood, and young adulthood
• Both males and females are affected
• A vast majority of cases are seen among fair-skinned Afrikaners; the incidence rate is around 1 in 7,200 among this population
• A few rare cases have been reported from northwestern European nations such as Denmark and Germany

What are the Risk Factors for Keratolytic Winter Erythema? (Predisposing Factors)

The risk factors for Keratolytic Winter Erythema (KWE) include:

• A positive family history of KWE
• Children born to consanguineous partners or spouses are noted to have a higher risk for the condition
• Individuals belonging to Oudtshoorn (Afrikaner) descendants

The following factors are known to worsen KWE:

• Winter season
• Increased exposure to the following:
  • Chemicals
  • Friction
  • Water
• Certain medication use such as topical steroids
• Emotional and psychological stress
• Menstruation
• General anesthesia
• Illnesses
• Superimposed bacterial infections

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Keratolytic Winter Erythema? (Etiology)

Keratolytic Winter Erythema is described as a form of genodermatosis, a hereditary skin condition. It is caused by genetic mutations located on chromosome 8p.

• The mutations causing the condition are inherited in an autosomal dominant manner
• Very few sporadic cases have been reported, meaning there has been no preceding family history of the condition in some individuals

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition, or pass it on to their offspring.

What are the Signs and Symptoms of Keratolytic Winter Erythema?

The redness and associated peeling of skin in Keratolytic Winter Erythema (KWE) are known to cyclically repeat in an intermittent manner; however, the severity of the condition may vary from time to time. In general, the signs and symptoms include:

• Presence of thickened skin with redness and irritation; the redness may be circular in shape
• The peeling of skin takes place after the reddish discoloration is noted; this occurs cyclically over several decades
• Air-filled dry blisters with opaque skin are noted on the superficial areas, which then rupture to result in skin peeling
• The peeling is often irregular, and peeling takes place from multiple sites, typically proceeding from the center of the palm/sole to the outside
• The palms of the hands and soles of the feet are commonly affected; the entire palm and sole surface, including the webbing between the digits may be involved
• In severe cases, the condition involves the arms and legs, buttocks, trunk region, and extremely rare, the face
• The peeling of skin is typically painless; the skin patch area that peels forms and progresses over 4-6 weeks
• In individuals with sweating of the palms/soles, smelly hands or feet may be noted
• Sweating is known to increase the rate of peeling

The onset of the condition begins in winter; the cold season also aggravates KWE and makes it more pronounced. However, an improvement may be noted during summers. Also, in pregnant women, the condition may be less severe.

How is Keratolytic Winter Erythema Diagnosed?

A diagnosis of Keratolytic Winter Erythema may involve the following exams and procedures:

• Physical examination of the individual and medical history evaluation, including evaluating one’s family history
• Assessment of the presenting signs and symptoms
• Blood tests, analysis of pus, culture of the fluid being drained, as needed
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms such as:

• Annular erythema
• Circumscribed palmar hypokeratosis (CPH)
• Erythrokeratoderma
• Keratolysis exfoliativa
• Localized epidermolysis bullosa simplex
• Palmoplantar keratoderma (PPK)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Keratolytic Winter Erythema?

The possible complications due to Keratolytic Winter Erythema include:

• Severe emotional stress due to cosmetic issues
• In some individuals, an acute flaring up of the symptoms can severely affect one’s quality of life
• Superimposed bacterial and fungal skin infections
• Cyclical recurrence of the condition and lifelong persistence
• Treatment complications including resistance to conventional therapies

How is Keratolytic Winter Erythema Treated?

There is neither any cure nor any no well-defined treatment measures available for Keratolytic Winter Erythema. The healthcare provider may consider the following treatment measures:

• Use of topical keratolytics containing salicylic acid, coal tar, or urea, may be helpful in some; while in others, it may worsen the condition
• Application of topical steroids may be helpful, but is usually a temporary solution
• Photodynamic therapy (PDT)

Regular follow up visits with the healthcare provider is important and recommended.

How can Keratolytic Winter Erythema be Prevented?

Currently, Keratolytic Winter Erythema may not be preventable since it is a genetic disorder.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child, particularly if the condition is accompanied by additional signs and symptoms such as reduced sweat glands 
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

Avoiding the exacerbating or triggering factors is important to control severity of the condition.

What is the Prognosis of Keratolytic Winter Erythema? (Outcomes/Resolutions)

The prognosis of Keratolytic Winter Erythema (KWE) is generally good in cases that present mild signs and symptoms.

• The condition is known to get better with age. It is also known to improve during summers
• In severe cases, KWE can be a recurrent and longstanding condition, often persisting for many years and decades

Additional and Relevant Useful Information for Keratolytic Winter Erythema:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/