Acropustulosis of infancy L44.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.01.2024

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Synonym(s)

acropustulosis infantilis; acropustulosis of infancy; infantile acropustulose; Infantile acropustulose; infantile acropustulosis

History
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Kahn and Rywlin, 1979

Definition
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Rare, usually intensely itchy, intermittent (flare-up duration 2-3 weeks), time-limited sterile pustulosis of unknown origin occurring on the palms of the hands and soles of the feet. Episodic course with no symptoms for months. Spontaneous healing usually between the ages of 2 and 4.

Etiopathogenesis
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Unexplained; possible triggers are atopic diathesis and scabies (persistent immune reaction in a previous scabies infection).

Manifestation
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Usually begins in the first months of life, manifestation peak in infants and toddlers, rarely connatal.

Localization
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Palms of hands, soles of feet; also on the dorsal sides of hands and feet. Rarely, and then only sporadically, pustulation of the trunk, buttocks or capillitium.

Clinical features
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Disseminated, inflammatory reddened papules rapidly transform into papulovesicles and finally into pustules and subsequently into crusty lesions. No confluence. Regression within 1-2 weeks. Severe itching. Chronic recurrent course with disease careers of up to 3 years. In the last phase of the disease, the intervals without appearance are prolonged, while at the same time the acuteity of the disease flattens.

Histology
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Intraepidermal, mainly subcorneal, unilocular pustules with neutrophilic, sometimes also eosinophilic granulocytes. Perivascular round cell infiltrate in the upper dermis.

Diagnosis
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Clinical signs (acral localization, severe itching, intermittent course) and medical history are diagnostic.

Differential diagnosis
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the most important differential diagnosis is superinfected scabies (mite detection necessary for differentiation)!

Further differential diagnoses:

Therapy
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Self-limiting course; causal therapy not known. Parents should be informed in detail about benignity and the particularities of the clinical course.

External therapy
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Symptomatic, e.g. astringent shaking mixtures or ointments with oak bark or synthetic tanning agents (e.g. Tannolact cream, Tannosynt Lotio).

If the pustulation is very pronounced, application of weakly effective (possibly under-dosed) external glucocorticoids such as 0.5% hydrocortisone cream.

Alternatively a 2-3% polidocanol cream should be used.

Internal therapy
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Therapy with DADPS (1.0-3.0 mg/kg bw/day) successful, but only to be used with a self-limited clinical picture under strict indication (Met-Hb formation, haemolysis!). Recurrence at weaning.

Progression/forecast
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Spontaneous healing between the ages of 2 and 4.

Case report(s)
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The 7-month-old infant suffered since the 2nd month of life from relapsing-like pustular formations on both soles of the feet. The parents reported an agonizing itching, which regularly affected their postpartum rest. All local measures had been unsuccessful so far.

Findings: On both soles of the feet disseminated and grouped vesicles of 0.1-0.3 cm in size and pale yellow pustules on bright red erythema and plaques. Older lesions were characterized by a coarse lamellar scaling.

Diagnosis: No evidence of scabies. Pustule smear: Sterile. Histology: Detection of subcorneal unilocular pustules with neutrophil, sometimes eosinophilic granulocytes. Bulky, perivascular round cell infiltrate in the upper dermis.

Therapy: 0.5% hydrocortisone cream alternating with a 3% polidicanol cream.

Course: Further relapsing activities until 18 months of age. Afterwards, the relapses subside for another 3 months and healing takes place.

Literature
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  1. Braun-Falco M et al (2003) Palmoplantar vesicular lesions in childhood. Dermatologist 54: 156-159
  2. Ferreira S et al (2020) Infantile acropustulosis. J Paediatr Child Health 56:1165-1166.
  3. Good LM et al (2011) Infantile acropustulosis in internationally adopted children. J Am Acad Dermatol 65:763-771
  4. Hürlimann AF, Wüthrich B (1992) Infantile acropustulosis. Z Hautkr 67: 1073-1079
  5. Kahn G, Rywlin AM (1979) Acropustulosis of infancy. Arch Dermatol 115: 831-833
  6. Klein CE et al (1989) Infantile acropustulosis. Dermatologist 40: 501-503
  7. Mazereeuw-Hautier J (2004) Infantile acropustulosis. Press Med 33:1352-1354.
  8. Meiss F et al (2008) Infant with pustules on both plantae. Dermatology 59: 323-324
  9. Paloni G et al (2013) Acropustulosis of infancy. Arch Dis Child Fetal Neonatal Ed 98:F340.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 09.01.2024