11 / 20
Volume 4
Journal of Pediatric Care
ISSN: 2471-805X
Page 39
JOINT EVENT
Pediatric Critical Care 2018 &
World Pediatrics 2018
October 18- 20 , 2018
October 18- 20, 2018 Warsaw, Poland
&
6
th
International Conference on
25
th
World Pediatrics Conference
Pediatric Critical Care and Emergency Medicine
Arare case of linear and whorled nevoid hypermelanosis with global developmental delay, scoliosis and
retinal degeneration with dermoscopic features
Deo Adiel Wong
Jose R Reyes Memorial Medical Center, Philippines
Only 40 cases of the rare sporadic linear and whorled nevoid hypermelanosis (LWNH) are described in medical literature
worldwide. It is characterized by hyperpigmented, reticulated, streaky and whorled patches along Blaschko’s lines, without
atrophy or preceding inflammation. It reflects an underlying mosaicism and is occasionally associated with systemic
abnormalities. A five year-old female presented in our institution with multiple uniformly hyperpigmented patches, some
linear over right upper extremity, both lower extremities and trunk with midline demarcation. Lesions were unchanged and
asymptomatic since birth. Dermoscopy showed linear brown streaks with alignment along Blaschko’s lines over right upper
extremity, reticular over right thigh and brown structure less zones interrupted by dotted perifollicular hypopigmentation over
right posterior trunk and anterior thigh. Histopathology revealed basal layer hyperpigmentation, sparse superficial lymphocytic
dermal infiltrates, melanocytic hypermelanosis and flat-topped papillomatosis. Hematological and biochemical tests revealed
no abnormalities. The patient has decompensated thoracic scoliosis with 2 cm divergence from plumb line. Developmental
pediatrics referral revealed assessment of global developmental delay and examination under anesthesia showed peripheral
retinal degeneration on both eyes. While this rare occurrence presents a challenging situation and is one reason why data is
lacking, affected individuals should be evaluated because of disease associations, including a search for developmental and
growth delays, skeletal abnormalities, and other systemic abnormalities. Despite few associations from previous case reports,
no promise of chromosomal abnormalities can be found using next generation or whole exome sequencing. Together with the
fact that treatments have been tried without much success, makes it a necessity for reporting.
deoadielwong@gmail.comJ Pediatr Care 2018, Volume 4
DOI: 10.21767/2471-805X-C4-015