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

• Acrokeratosis Verruciformis
• AVH (Acrokeratosis Verruciformis of Hopf)

What is Acrokeratosis Verruciformis of Hopf? (Definition/Background Information)

• Acrokeratosis Verruciformis of Hopf (AVH) is a rare, benign skin condition that is characterised by wart-like papules on the hands and feet. It is a genetic disorder that is usually manifested at or following the birth of the child
• The condition is known to be caused by genetic mutations, with involvement of the ATP2A2 gene. A family history of Acrokeratosis Verruciformis of Hopf is a key risk factor
• A diagnosis of Acrokeratosis Verruciformis of Hopf can be made by clinical exam, skin exam, and various other diagnostic tools including dermoscopy and wood’s lamp examination
• If the symptoms are mild, then treatment may not be necessary. In case of significant symptoms, the treatment may involve the use of moisturizers, topical creams, and skin softeners
• The prognosis of Acrokeratosis Verruciformis of Hopf depends upon the severity of the symptoms. Generally, with suitable treatment, the prognosis is good

Who gets Acrokeratosis Verruciformis of Hopf? (Age and Sex Distribution)

• Acrokeratosis Verruciformis of Hopf is a congenital skin condition. It may be observed at birth or during early childhood
• The condition affects both males and females
• It is seen worldwide and all racial and ethnic groups may be affected

What are the Risk Factors for Acrokeratosis Verruciformis of Hopf? (Predisposing Factors)

• The main risk factor for Acrokeratosis Verruciformis of Hopf is a positive family history of AVH

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 Acrokeratosis Verruciformis of Hopf? (Etiology)

Acrokeratosis Verruciformis of Hopf is a genetic disorder with a hereditary origin, in some cases. It is caused by genetic mutations that are passed down through families in an autosomal dominant pattern.

• The gene involved is ATP2A2, for inherited forms of AVH
• Chromosomal abnormalities on 12q24.11 are noted
• These mutations are also seen in Darier disease, and hence, AVH is believed to be a subtype of Darier disease (another autosomal dominant skin condition)

In some cases, the condition occurs in a sporadic manner, indicating that a prior family history is not present.

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 Acrokeratosis Verruciformis of Hopf?

The signs and symptoms associated with Acrokeratosis Verruciformis of Hopf may include:

• The presence of wart-like papules in the hands/feet
• The papules are mostly grouped into small areas on the skin
• Pitting of skin of the palms and soles
• Symmetrical lesions are present on the back of the hands/feet. Rarely, it can also affect the elbows and knees
• An injury to the affected skin may cause blister formation
• Changes in the nail (nail dystrophy); whitish discoloration of nail along with prominent ridges
• The severity of the symptoms may vary from one individual to another. Even within families (i.e., among family members), the severity may vary

How is Acrokeratosis Verruciformis of Hopf Diagnosed?

The diagnosis of Acrokeratosis Verruciformis of Hopf may involve:

• A complete evaluation of medical history (including family medical history) along with a thorough examination of the skin lesions by a dermatologist
• Dermoscopy: It 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
• Genetic testing to identify the gene involved
• Skin biopsy: A skin 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

Note: A skin biopsy may show characteristic features including “church spire” of the epidermis.

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 Acrokeratosis Verruciformis of Hopf?

Complications associated with Acrokeratosis Verruciformis of Hopf may include the following:

• Emotional stress due to the skin condition
• Cosmetic concerns

How is Acrokeratosis Verruciformis of Hopf Treated?

It is generally difficult to treat Acrokeratosis Verruciformis of Hopf. For mild cases, no treatment may be required. The skin symptoms may be addressed through treatment measures such as:

• Topical application of keratolytics (creams containing salicylic acid or urea)
• Use of moisturizing creams and lotions

How can Acrokeratosis Verruciformis of Hopf be Prevented?

Currently, Acrokeratosis Verruciformis of Hopf is a genetic disorder that cannot be prevented. However, the following may be considered:

• 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
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as AVH

What is the Prognosis of Acrokeratosis Verruciformis of Hopf? (Outcomes/Resolutions)

The prognosis of Acrokeratosis Verruciformis of Hopf may depend on the severity of the signs and symptoms. In most cases, the prognosis is good with appropriate treatment.

• However, in general, AVH is difficult to treat and may present treatment challenges
• Regular follow up visits with the healthcare provider are important and encouraged

Additional and Relevant Useful Information for Acrokeratosis Verruciformis of Hopf:

Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition.