Acropustulosis of Infancy 

Updated: Apr 15, 2019
Author: Cassondra A Ellison, MD; Chief Editor: Dirk M Elston, MD 

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 2-12 months of life (see the image below). First described in 1979, infantile acropustulosis is probably much more common than the scarcity of reports would imply.[1, 2]

Lateral and plantar aspects of the foot with a com Lateral and plantar aspects of the foot with a combination of intact acute vesicles and brownish hyperpigmentation of old vesicles.

Pathophysiology

The pathophysiology and etiology of infantile acropustulosis are 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, 6] The condition is fairly common among children adopted from foreign countries, a setting where scabies might be suspected.[7] 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 (atopy) has been noted.[7, 8]

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.[9] This should be distinguished from localized infantile acropustulosis.

Etiology

The cause of infantile acropustulosis is unknown. Scabies as a preceding or concomitant infestation in the same patient is well-documented in some cases. Many children are undoubtedly misdiagnosed with scabies and initially treated with lindane or permethrin without any confirmatory scrapings. It has been postulated that infantile acropustulosis is a cutaneous hypersensitivity reaction to antigens associated with scabies infestation.[10] No other infectious agent has been documented. Clinical response to a therapeutic trial of dapsone has been suggested to help differentiate between a scabetic and nonscabetic cause of the eruption; however, more data are needed.[11]

Some studies suggest that acropustulosis and neonatal eosinophilic pustular folliculitis are associated and may be manifestations of the same underlying disease.[12, 13]

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.[14]

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, 14]

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.[14] Resolution by age 3 years is the norm.

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, with definite resolution by age 9 years.

 

Presentation

History

The classic history of infantile acropustulosis is an infant aged 2-12 months developing recurrent crops of pruritic erythematous macules or papules that progress into vesicles and then pustules. Lesions typically follow this progression over the course of 24 hours. Onset is usually in the first 3 months of life but lesions may sometimes be present at birth.[15] Children are fretful, irritable, and obviously uncomfortable, but otherwise healthy. Lesion are intensely pruritic and individual bouts of infantile acropustulosis last 3-14 days and recur in 2- to 4-week intervals. The attacks occur with progressively diminishing numbers of lesions, and with decreasing frequency, until they cease altogether. This typically occurs within 2 years of onset.[10, 15]

Often, children have been empirically treated with antiscabies medicines prior to presentation,[5] and some have been treated with topical or oral antibiotics as well. The intensity and the duration of infantile acropustulosis attacks diminish with each recurrence. Children with acropustulosis may be misdiagnosed with bacterial infections, hand-foot-and-mouth disease (Coxsackievirus or Enterovirus infection), or dyshidrotic eczema.[7]

Physical Examination

Acropustulosis of infancy is characterized by recurrent outbreaks. Lesions begin as crops of small macules or papules that eventually form distinct, noncoalescing vesicles and pustules. Lesions occur in an acral distribution, usually involving the palms, soles, and lateral surfaces of the digits.[15] Less frequently, lesions may occur on the dorsal hands, feet, trunk, scalp, and face.

They resolve with desquamation followed by postinflammatory macular hyperpigmentation. No other organ systems are involved.

See the images below.

Lateral and plantar aspects of the foot with a com Lateral and plantar aspects of the foot with a combination of intact acute vesicles and brownish hyperpigmentation of old vesicles.
Pustules on the dorsal hands of a 1-year-old child Pustules on the dorsal hands of a 1-year-old child.
Scattered new and resolving pustules on the dorsal Scattered new and resolving pustules on the dorsal feet.

Complications

Secondary bacterial infection of excoriated lesions may occur.

 

DDx

Diagnostic Considerations

For an infant presenting with a pustular vesicular dermatitis, disease considerations may be separated into infectious and noninfectious categories. Noninfectious etiologies include erythema toxicum neonatorum, acne neonatorum, transient neonatal pustular melanosis, eosinophilic pustular folliculitis, mastocytosis, malaria neonatorum, acne neonatorum, and incontinentia pigmenti. Infectious processes to be considered include scabies, bacterial sepsis, impetigo, staphylococcal scalded skin syndrome, cutaneous candidiasis, herpes simplex infection, syphilis, and varicella infection.[16]

Differential Diagnoses

 

Workup

Laboratory Studies

No laboratory studies are needed to make the diagnosis of infantile acropustulosis, as the diagnosis typically is clinical. A complete blood cell count often shows eosinophilia. Cultures and smears are sterile and help to rule out an infectious etiology. They show predominance of eosinophils and later neutrophils.[15]

Histologic Findings

A unilocular, subcorneal, or intraepidermal pustule containing polymorphonuclear neutrophils or eosinophils in the upper epidermis and extending into the stratum corneum is characteristic in infantile acropustulosis. Papillary dermal edema and a mild perivascular, mostly lymphocytic, infiltrate in the dermis may be present.[10, 14] Direct immunofluorescence results are negative.

 

Treatment

Medical Care

Acropustulosis of infancy is generally unresponsive to therapy; however, it is self-limited.

Topical pramoxine preparations are available without prescription for the treatment of pruritus. Sedating oral antihistamines (eg, hydroxyzine, diphenhydramine) may be useful in infantile acropustulosis to help decrease pruritus, especially at night.

Treatment to relieve the pruritus can be helpful. Topical steroids[5] and oral dapsone[17] have been used successfully, if justified in more difficult cases. As the condition may be difficult to differentiate from scabies, a therapeutic treatment trial may be indicated.[15]

Appropriate workup for glucose-6-phosphate dehydrogenase (G6PD) deficiency should be considered before prescribing oral dapsone, owing to the risk of hemolysis.[18]

Limited case reports describe topical maxacalcitol being useful for management, but more research is required.[19]

Topical or oral antibiotics should not be given unless the lesions become secondarily infected with bacteria.

Consultations

In infantile acropustulosis, consult a dermatologist or a pediatric dermatologist.

Activity

No activity changes are necessary. Isolation is not warranted.

Diet

No dietary changes are necessary.

 

Medication

Medication Summary

High-potency topical steroids (classes 1 and 2) have been used successfully for control of pruritus. Oral sedating antihistamines may reduce pruritus and aid in sleep for irritable and extremely uncomfortable children. Children who are extremely symptomatic may be treated with dapsone as it has been noted to relieve symptoms within 24 hours and signs within 72 hours in some cases.[15] Oral antibiotics and oral glucocorticoids have been used in isolated cases, but are rarely necessary.[10]

Topical steroids

Class Summary

These agents provide symptomatic relief of pruritus.

Betamethasone topical (Diprolene, Betatrex)

Topical betamethasone is used for inflammatory dermatoses responsive to steroids. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability. Use fluorinated topical steroids with caution in children.

Antihistamines, 1st Generation

Class Summary

Sedating antihistamines may provide symptomatic relief of pruritus.

Diphenhydramine (Alka-Seltzer Plus Allergy, Benadryl, Benadryl Allergy Dye-Free LiquiGels)

Diphenhydramine is an antihistamine used for pruritus and allergic reactions.

Hydroxyzine (Vistaril)

Hydroxyzine is an antihistamine with antipruritic, anxiolytic, and mild sedative effects. It antagonizes H1 receptors in the periphery, and it may suppress histamine activity in the subcortical region of the CNS. Syrup is available as 10 mg/5 mL.

Antibiotics

Class Summary

Diaminodiphenylsulfone antibiotics have been used as anti-inflammatory agents.

Dapsone (Avlosulfon)

Dapsone is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to that of sulfonamides, where competitive antagonists of PABA prevent the formation of folic acid, inhibiting bacterial growth. It is used mainly to treat leprosy and dermatitis herpetiformis. Dapsone has antineutrophil and anti-inflammatory properties.

Antipruritics

Class Summary

These agents may relieve associated itching.

Pramoxine topical (Tronothane, Prax)

Topical pramoxine blocks nerve conduction and impulses by inhibiting depolarization of neurons. Use 1% lotion or cream.