Acropustulosis of Infancy

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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]



View Image

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.

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.



View Image

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



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Pustules on the dorsal hands of a 1-year-old child.



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Scattered new and resolving pustules on the dorsal feet.

Complications

Secondary bacterial infection of excoriated lesions may occur.

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.

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 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]

Betamethasone topical (Diprolene, Betatrex)

Clinical Context:  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.

Class Summary

These agents provide symptomatic relief of pruritus.

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

Clinical Context:  Diphenhydramine is an antihistamine used for pruritus and allergic reactions.

Hydroxyzine (Vistaril)

Clinical Context:  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.

Class Summary

Sedating antihistamines may provide symptomatic relief of pruritus.

Dapsone (Avlosulfon)

Clinical Context:  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.

Class Summary

Diaminodiphenylsulfone antibiotics have been used as anti-inflammatory agents.

Pramoxine topical (Tronothane, Prax)

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

Class Summary

These agents may relieve associated itching.

Author

Cassondra A Ellison, MD, Resident Physician, Department of Dermatology, Geisinger Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Barbara B Wilson, MD, Edward P Cawley Associate Professor, Department of Dermatology, University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Nicole Lee Edmonds, University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Vernon J Forrester, MD, Resident Physician, Department of Dermatology, UVA Health System, University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Michael J Wells, MD, FAAD, Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Disclosure: Nothing to disclose.

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Daniel Mark Siegel, MD, MS, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Howard Pride, MD Associate Physician, Departments of Pediatrics and Dermatology, Geisinger Medical Center

Howard Pride is a member of the following medical societies: American Academy of Dermatology and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

References

  1. Kahn G, Rywlin AM. Acropustulosis of infancy. Arch Dermatol. 1979 Jul. 115(7):831-3. [View Abstract]
  2. Zuniga R, Nguyen T. Skin conditions: common skin rashes in infants. FP Essent. 2013 Apr. 407:31-41. [View Abstract]
  3. Humeau S, Bureau B, Litoux P, Stalder JF. Infantile acropustulosis in six immigrant children. Pediatr Dermatol. 1995 Sep. 12(3):211-4. [View Abstract]
  4. Prendiville JS. Infantile acropustulosis--how often is it a sequela of scabies?. Pediatr Dermatol. 1995 Sep. 12(3):275-6. [View Abstract]
  5. Mancini AJ, Frieden IJ, Paller AS. Infantile acropustulosis revisited: history of scabies and response totopical corticosteroids. Pediatr Dermatol. 1998 Sep-Oct. 15(5):337-41. [View Abstract]
  6. Chiu Y, Humphrey S. Congenital cutaneous lesions and infantile rashes. Bordini BJ, Basel D, Toth H, Lye PS, Kliegman R, eds. Nelson Pediatric Symptom-Based Diagnosis. 1st Ed. Elsevier; 2018. 851-65.
  7. Good LM, Good TJ, High WA. Infantile acropustulosis in internationally adopted children. J Am Acad Dermatol. 2011 Oct. 65(4):763-71. [View Abstract]
  8. Howard RM, Frieden IJ. Vesicles, pustules, bullae, erosions, and ulcerations. Eichenfield L, Frieden I, Mathes E, Zaenglein A, eds. Neonatal Dermatology and Infant Dermatology. 3rd Ed. Saunders; 2015. 111-39.
  9. Minkis K, Aksentijevich I, Goldbach-Mansky R, et al. Interleukin 1 receptor antagonist deficiency presenting as infantile pustulosis mimicking infantile pustular psoriasis. Arch Dermatol. 2012 Jun. 148(6):747-52. [View Abstract]
  10. Infantile acropustulosis. Larralde M, Boldrini MP, Luna PC, Abad ME, Marín CC. Dermatología Argentina. 2013. 16(2010):268-71.
  11. Truong AL, Esterly NB. Atypical acropustulosis in infancy. Int J Dermatol. 1997 Sep. 36(9):688-91. [View Abstract]
  12. Tucker M, Ramolia P, Wells MJ. JAAD Grand Rounds. Neonate with extensive papulovesicles. J Am Acad Dermatol. 2013 May. 68 (5):877-9. [View Abstract]
  13. Vicente J, España A, Idoate M, Iglesias ME, Quintanilla E. Are eosinophilic pustular folliculitis of infancy and infantile acropustulosis the same entity?. Br J Dermatol. 1996 Nov. 135 (5):807-9. [View Abstract]
  14. Dromy R, Raz A, Metzker A. Infantile acropustulosis. Pediatr Dermatol. 1991 Dec. 8(4):284-7. [View Abstract]
  15. Ghosh S. Neonatal pustular dermatosis: an overview. Indian J Dermatol. 2015 Mar-Apr. 60 (2):211. [View Abstract]
  16. DeLeon SD, Melson SC, Yates AB. Crawling Toward a Diagnosis: Vesicles and Thrombocytopenia in a Neonate. Hosp Pediatr. 2015 Oct. 5 (10):555-7. [View Abstract]
  17. Truong AL, Esterly NB. Atypical acropustulosis in infancy. Int J Dermatol. 1997 Sep. 36(9):688-91. [View Abstract]
  18. Silverberg NB. Infantile Acropustulosis. Silverberg NB, Durán-McKinster C, Tay YK, eds. Pediatric Skin of Color. Springer; 2015. 4(36): 323-25.
  19. Kimura M, Higuchi T, Yoshida M. Infantile acropustulosis treated successfully with topical maxacalcitol. Acta Derm Venereol. 2011 May. 91(3):363-4. [View Abstract]

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

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.

Scattered new and resolving pustules on the dorsal feet.

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.

Scattered new and resolving pustules on the dorsal feet.