Pustular Diseases of Early Childhood




Patient Story



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A 1-year-old boy is brought for a second opinion about the recurrent pruritic vesicles and pustules on his hands and feet. This is the third episode, and in both previous episodes, the physicians thought the child had scabies. The child was treated with permethrin both times and within 2 to 3 weeks the skin cleared. No other family members have had lesions or symptoms. Figures 94-1 to 94-3 demonstrate a typical case of infantile acropustulosis that is often misdiagnosed as scabies. Although the condition can be recurrent, it is ultimately self-limited and will resolve.




FIGURE 94-1


Infantile acropustulosis (acropustulosis of infancy) on the foot of a 1-year-old boy. (Used with permission from Richard P. Usatine, MD.)






FIGURE 94-2


Acropustulosis with vesiculopustular eruption on the toes of the boy shown in Figure 94-1. (Used with permission from Richard P. Usatine, MD.)






FIGURE 94-3


Acropustulosis with pruritic eruption on the hand and wrist of the boy shown in Figures 94-1 and 94-2. (Used with permission from Richard P. Usatine, MD.)






Introduction



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Acropustulosis and transient neonatal pustular melanosis (TNPM) are pustular diseases that typically present in infancy. Acropustulosis is a pruritic vesiculopustular disease presenting between 2 and 10 months of age and remitting spontaneously by 36 months of age. TNPM is present at birth and characterized by 2- to 3-mm hyperpigmented macules and pustules. Acropustulosis may require symptomatic treatment of pruritus, but otherwise both illnesses are self-limiting.




Epidemiology



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Acropustulosis:





  • Rare, intensely pruritic, vesiculopustular disease of young children.1



  • Typically begins in the second or third months1 of life and as late as 10 months of age.2



  • Occur slightly more often in darker-skinned patients and males.1



  • Typically spontaneously remits by 6 to 36 months of life.2




Transient neonatal pustular melanosis:





  • A disease of newborns.3



  • Equal male-to-female ratio.3



  • Seen in 4.4 percent of black infants and 0.6 percent of white infants.4



  • Early, spontaneous remission.3





Etiology and Pathophysiology



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Acropustulosis:





  • The exact cause and mechanism have yet to be determined.5



  • Some physicians speculate that it is a persistent reaction to scabies (“postscabies syndrome”). Suggestive of this infectious etiology, infantile acropustulosis will occasionally be concurrently present among siblings. Also, patients diagnosed with this disorder frequently have received prior treatment for scabies, which may either provide evidence of an infectious etiology or demonstrate the frequent misdiagnosis, as in the previous patient. Odom et al. concludes that in some cases, this disease may represent a hypersensitivity reaction to Sarcoptes scabiei.4




Transient neonatal pustular melanosis:





  • The etiology is uncertain;6 however, it may result from an obstruction of the pilosebaceous orifice.3





Diagnosis



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  • Acropustulosis—A workup to rule out potentially serious infectious causes should be considered whenever confronted with a new pustular dermatosis early in a child’s life. A workup might include a scraping for scabies and KOH preparation as rapid diagnostic tests. If these studies are negative, the diagnosis may be made clinically as described below.



  • TNPM—This diagnosis can often be made clinically. However, if performed, a Wright stain of the exudate will reveal a predominance of neutrophils with an occasional eosinophil, and the Gram stain will be negative.3


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Dec 31, 2018 | Posted by in PEDIATRICS | Comments Off on Pustular Diseases of Early Childhood

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