PracticeUpdate Dermatology Best of 2018

EXPERT OPINION 22

Pearls From the Fall Clinical Dermatology Meeting 2018: Dr. Albert Yan By Albert C. Yan MD Dr. Yan is Chief of Pediatric Dermatology at the Children’s Hospital of Philadelphia, Professor of Pediatrics and Dermatology at Perelman School of Medicine at the University of Pennsylvania in Philadelphia, Pennsylvania.

in children: localized palmar erythema of adenovirus gastroenteritis 3 as well as adenovirus-associated annular eruptive pseudoangiomatosis, wherein patients affected have a prolonged, weeks-long eruption characterized by discrete small more typical pseudoangiomatous papules and concomitant annular lesions. 4 Severe adenovirus infections can also be seen in patients undergoing stem cell transplantation, and these infections are associated with mortality rates as high as 75% in those affected due to disseminated multiorgan infections. 5 Small stains with big implications Small coin-sized capillary malformations with surrounding blanching are hallmarks of capillary malformation–arteriovenous malformation (CM-AVM) syndrome, a geno- dermatosis associated with visceral AVMs, particularly of the central nervous system. In this way, it resembles hereditary hemor- rhagic telangiectasia syndrome. Although 70% of those with CM-AVM have mutations in the RASA1 gene, which regulates vascu- lar growth through the RAS-MAPK pathway, it has been suspected that other genes within this pathway might also produce similar phenotypes. Recently, two papers highlighted a new gene, EPHB4, which is responsible for a subset and milder pheno- type of CM-AVM syndrome and is referred to as CM-AVM 2 syndrome. 6,7 EPHB4 func- tions in the same RAS-MAPK pathway, although it is further upstream. Multiple cutaneous hemangiomas represent a skin marker for having visceral hemangi- omatosis, particular of the liver. Although it has long been thought that typical multifocal hepatic hemangiomas behave and respond like cutaneous hemangiomas, a disturbing development was highlighted in a series of eight cases of typical liver hemangiomas (glut-1–positive, histologically consistent infantile hemangiomas, responding to pro- pranolol to inhibit growth) that evolved into angiosarcoma. 8 These few cases appear to be extremely rare, but suggest that

Pediatric dermatology update: What’s new?

New developments in the care of pediatric skin disease have come to light over the past year that have clinical relevance for the practicing dermatologist. Fighting enamel cruelty Because dental enamel maturation is not completed until 8 years of age, clinicians typically avoid use of tetracycline deriva- tives due to concerns about permanent staining of the enamel. However, mount- ing clinical experience in using tetracycline derivatives in children for indications such as malaria, Rocky Mountain spotted fever, and Lyme disease have shown no signif- icant staining in children. In an article by Pöyhönen et al in the Journal of Antimi- crobial Chemotherapy, for instance, 38 children under the age of 8 years received doxycycline for up to 28 days for vari- ous indications. 1 In their series, no dental enamel staining was noted in any of the children. The current edition of the Amer- ican Academy of Pediatrics Red Book (2018–2021) states that doxycycline can be used in children without regard to patient age if used for 21 days or fewer. Going viral Parechovirus infection represents one of the most common causes of bacterial culture-negative neonatal fever. Children with this infection may present with fever, exanthem with notable acral accentuation (hands and feet as well as the wrists and ankles). Affected neonates are often irri- table as well, earning the moniker of “hot, angry baby” syndrome due to the fever, exanthem, and meningoencephalitis pic- ture that characterizes this infection in newborns. 2 Adenovirus is known to cause an infectious syndrome that can at times resemble Kawa- saki disease due to the associated fever, conjunctivitis, exanthem, and acral accen- tuation. Two sets of papers highlight some other notable presentations of adenovirus

children with liver hemangiomas should be monitored to make sure lesions regress completely and show no signs of recurrence during childhood. Cases of angiosarcoma typically presented by 3 years of age; so, it may be prudent to re-evaluate these chil- dren again at that age. A slime of the times Homemade slime is a gelatinous semisolid compound made from school glue, sodium borate, and water that can be made at home. Schoolchildren often make this con- coction with glitter, food coloring, or other additives to personalize their homemade slime. Parents often support this activity as a home science experiment, and kids are known to play with their slime kneading it and mashing it for up to several hours at a time. Since 2017, several reports have emerged initially in the media and more recently in the medical literature describing mild to severe irritant chemical burn–like reactions after several hours of exposure. 9 Occasionally, allergic contact reactions have been identified to methylisothiazoli- none (MI) and methylchloroisothiazolinone (MCI) in the glue used in the slime as con- firmed by patch testing. 10 The presence of MI/MCI was verified by mass spectrometry. Hope for epider molysis bullosa A report in the journal Nature documented the dramatic success of transgenic skin grafts for the treatment of severe junctional epidermolysis bullosa (JEB). 11 A critically ill 7-year-old boy with severe JEB was treated with autologous transgenic keratinocyte skin grafts, which were originally derived from an unaffected area of skin that underwent retroviral gene transfer of a corrected laminin-332 gene. The grafts were simply laid directly onto denuded skin areas and successfully engrafted. The child experienced sustained improvement and correction of the defective epidermis through the proliferation of stem cell

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