Acropustulosis of Infancy

Updated: Jun 27, 2016
  • Author: Cassondra M Andreychik, MD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Infantile acropustulosis is a recurrent, self-limited, pruritic, vesicopustular eruption of the palms and the soles occurring in young children during the first 2-3 years of life. First described in 1979, infantile acropustulosis is probably much more common than the scarcity of reports would imply. [1, 2]

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Pathophysiology

The pathophysiology of infantile acropustulosis is unknown. Many cases of infantile acropustulosis are preceded by well-documented or suspected scabies infestation, and a scabies id reaction has been suggested. [3, 4, 5] The condition is fairly common among children adopted from foreign countries, a setting where scabies might be suspected. [6] More often, cases of infantile acropustulosis occur despite scabies having been thoroughly ruled out.

Bacterial and viral culture results are consistently negative, and negative immunofluorescence results suggest that infantile acropustulosis is not an antibody-mediated autoimmune process.

An association with atopic dermatitis has been noted. [6]

The most recently recognized cause of infantile pustulosis is a deficiency of interleukin 1 receptor antagonist (DIRA), which is a recessive syndrome caused by a loss of function mutation resulting in unopposed action of interleukin 1 and life-threatening systemic inflammation. [7] This should be distinguished from localized infantile acropustulosis.

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Epidemiology

Frequency

The exact incidence of infantile acropustulosis is unknown. One study from Israel reported 25 cases of infantile acropustulosis in a 9-year period, suggesting that this is not as uncommon as once thought. [8]

Race

Early reports suggested a predominance of African Americans for infantile acropustulosis. Now, acropustulosis of infancy is believed to affect all races equally.

Sex

Early reports suggested a male predominance for infantile acropustulosis. Larger series have since shown an equal distribution between males and females. [5, 8]

Age

Although children as old as 9 years have been reported to have infantile acropustulosis, acropustulosis of infancy typically begins between the first 2-12 months of life. [8] Resolution by age 3 years is the norm.

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Prognosis

Prognosis is for infantile acropustulosis excellent. Generally, the bouts of pruritic vesicopustules decrease in severity with successive outbreaks and resolve by age 2-3 years.

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