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

• PP (Parakeratosis Pustulosa)

What is Parakeratosis Pustulosa? (Definition/Background Information)

• Parakeratosis Pustulosa (PP) is a pediatric skin condition of unknown cause affecting a finger or toe resulting in nail deformity and other associated abnormalities. The condition may be confused with atopic eczema or nail psoriasis 
• The condition causes nail deformity including the loss of nail. Parakeratosis Pustulosa may be treated using topical creams and ointments. The condition is known to resolve spontaneously in many children

Who gets Parakeratosis Pustulosa? (Age and Sex Distribution)

• Parakeratosis Pustulosa is predominantly observed in young children
• Both males and females are affected, although girls are affected much more than boys (girl-boy ratio is 3:1)
• Worldwide, there is no racial or ethnic preference observed

What are the Risk Factors for Parakeratosis Pustulosa? (Predisposing Factors)

• Presently, no risk factors have been identified for Parakeratosis Pustulosa

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 Parakeratosis Pustulosa? (Etiology)

The exact cause of formation of Parakeratosis Pustulosa is presently not well-understood.

What are the Signs and Symptoms of Parakeratosis Pustulosa?

The signs and symptoms of Parakeratosis Pustulosa may include:

• Appearance of red skin on one side of the nail that progresses to involve the entire nail
• The skin around the nail may be red and scaly
• Typically, the condition involves a single digit - mostly the thumb or index finger is affected, but rarely, another finger/toe may also be involved
• Thickened skin beneath the further end of the nail, a condition referred to as subungual hyperkeratosis
• The thumb or index finger is swollen; broken cuticle or a loss of cuticle (the transparent dead skin at nail base) may be noted
• Onycholysis - lifting up or detachment of nail from nail bed that is often painless; this may be followed by shedding of the nail plate, called onychomadesis
• Pain and discomfort is common, but itching is not noted
• Other nail deformities may include pitting, transverse ridging, and blisters on fingertips

How is Parakeratosis Pustulosa Diagnosed?

A diagnosis of Parakeratosis Pustulosa may be difficult and challenging, as it can be commonly misdiagnosed for other skin conditions with similar features. The diagnosis may involve the following exams and procedures:

• Physical examination of the individual and medical history evaluation
• Blood tests and culture studies, 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, if necessary: 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:

• Acrodermatitis continua of Hallopeau
• Atopic dermatitis
• Hand dermatitis
• Nail psoriasis
• Paronychia
• Tinea pedis
• Tinea unguium

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 Parakeratosis Pustulosa?

The possible complications due to Parakeratosis Pustulosa include:

• Emotional stress
• Superimposed bacterial and fungal skin infections
• Development of psoriasis later in life
• Treatment complications

How is Parakeratosis Pustulosa Treated?

There are no well-defined treatment measures available for Parakeratosis Pustulosa. The healthcare provider may consider some of the following measures:

• Use of moisturizing creams
• Use of topical retinoids and steroids (high-potency)
• Application of calcipotriol ointment

How can Parakeratosis Pustulosa be Prevented?

Presently, it may not be possible to prevent Parakeratosis Pustulosa. However, the following may be considered to help children with the condition:

• Avoid thumb sucking
• Avoid playing in mud or other potentially infectious or inflammatory environments (reduce some outdoor play activities) till the condition improves or regresses
• Maintain good personal hygiene
• Wash the affected digit with water and mild antibacterial soap
• Avoid picking at the nail

What is the Prognosis of Parakeratosis Pustulosa? (Outcomes/Resolutions)

The prognosis of Parakeratosis Pustulosa is generally good as the condition improves over time.

Additional and Relevant Useful Information for Parakeratosis Pustulosa:

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

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