Infantile Acropustulosis

A vesicopustular rash in an infant

A 2-month old African American female presents to the dermatology office with a chief complaint of a rash for one month. The rash presented as flesh colored papules with central crust in a scattered distribution to the trunk, arms, and legs. Associated symptoms include pruritis and the patient’s mother also had a similar rash. A biopsy was performed which revealed scabies and the patient was treated with Permethrin 5% cream, which resolved the rash.

However, the patient reported to the dermatology office 10 days later with a new rash on the hands and feet that the patient’s mother described as pruritic. The new rash consisted of pustules to the dorsal and ventral surfaces of the hands and feet. All other body areas spared.

Differential Diagnosis

Hand foot mouth disease is a viral illness caused by the Cocksackie virus and less commonly Enterovirus. This rash typically presents with erythematous papules to the hands and feet. The lesions can also be present on the buttocks, arms, and legs. A flu-like prodrome often precedes the eruption.¹

Pustular psoriasis is a less common variant of psoriasis. It is uncommon in children, but can be triggered by medications, infection, systemic steroids, stress, and UV exposure. This rash presents with 1-3mm pustules on erythematous skin. It can be localized to the hands and feet or seen in a generalized form. The later may be accompanied by systemic symptoms such as headache, fever, and malaise and may be life-threatening.¹

Impetigo is a bacterial infection with the most common pathogens being Staphylococcus or Streptococcus species. It is characterized by blisters or sores most commonly located on the face, neck, hands, and diaper area. The characteristic lesions typically present with a yellow colored crust that may resemble honey or brown sugar.¹

Infantile acropustulosis is an intensely pruritic vesicopustular eruption on the hands and feet. The cause is unknown, but it is sometimes thought to be a hypersensitivity reaction to the scabies mite.¹

Diagnosis: Infantile Acropustulosis

Discussion

Infantile acropustulosis (IA) is recognized by an outbreak of 1-2 mm vesicles and pustules present primarily on both the hands and feet. Though the vesicopustules formed by IA are generally found in the acral areas of the hands and feet, they may appear on the scalp and trunk of the infant.

IA is further characterized by severe pruritis. This disease affects infants from the two to 24- month ranges, but may also be seen in children at the maximum of nine years of age. It is a rare condition and primarily affects infants with dark skin tones, but may occur in all races. This condition is self-limiting and will resolve in a few months to three years of age. During that time, however, it can reappear every three to four weeks with each occurrence typically lasting one to two weeks.1,3

The cause of IA is unknown, but many authors suggest a relationship between scabies and IA. This suggests a possibility of an idiopathic form and a hypersensitivity response to a previous infection by S. escabiei.⁵ Some cases have also shown atopic dermatitis as a precursor to IA.⁶

Pathology reveals an intraepidermal pustule or grouping of neutrophils. A positive biopsy will demonstrate subcorneal pustules of eosinophils in early lesions and higher percentage of neutrophils in later lesions. A smear may be used to detect and examine neutrophils and eosinophils to prevent misdiagnosis. No bacteria should be present in the smear to indicate that the pustules were created by IA rather than scabies infestation. The smear will also include focal degeneration of keratinocytes, dermal edema, and peripheral eosinophilia. A Gram’s stain can rule out viral infection.³

There is no cure for infantile acropustulosis, but treatment options include topical corticosteroids and oral antihistamines for symptom management. For recalcitrant cases, oral Dapsone is an option.

In this case, a culture was performed to rule out an infectious cause. Based on the clinical findings and history, the patient was diagnosed with infantile acropustulosis. Treatment consisted of fluocinolone oil to relieve the symptoms of pruritis. Unfortunately, the patient did not return for follow-up.^1,6

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References

1. Bolognia JL, et al. Dermatology. 2nd ed. New York, NY: Mosby Elsevier; 2008
2. Daly D, et al. A baby with blisters. Patient Care.1999: 205.
3. Kimura M, et al. Infantile acropustulosis treated successfully with maxacalcitol. Acta Dermato-Venereologica.2001;91:363-364. doi: 10.2340/00015555-1060
4. Larralde M, et al. Infantile acropustulosis. Dermatol Argent.2010;16(4):268-271 www.dermatolarg.org.ar/index.php/dermatolarg/article/download/…/574
5. Mancini A, Frieden l, Paller A. Infantile acropustulosis revisited: history of scabies and response to topical corticosteroids. Pediatric Dermatology.1998;15:337-341 doi: 10.1046/j.1525-1470.1998.1998015337
6. Silverberg N. Infantile acropustulosis. Pediatric skin of color.2015;4(36):323-325. doi10.1007/978-1-4614-6654-3_36.