Document Type : Case Report
Department of Pediatric Pulmonology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Neonatal and Children's Health Research Center, School of Medicine, Golestan University of Medical Sciences, Gorgan, Iran
Department of Radiology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Pediatric Pathology Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Student Research Committee, Lorestan University of Medical Sciences, Khorramabad, Iran.
Langerhans cell histiocytosis is an uncommon proliferative disorder that may influence many organs; so, the clinical presentations vary.
Here we describe an 85-day-old female who was born with In vitro fertilization after 10 years of infertility. She referred to us due to severe pulmonary insufficiency and congenital progressive maculopapular rash with desquamation. There were significant cystic changes in chest imaging studies. Further evaluation demonstrated lytic lesions in cranial, femoral, and humorous bones. The skin biopsy verified the diagnosis of LCH.
A combination of Vinblastine, VP16, and Dexamethasone regimen was applied for the patient. In the course of the disease, she encountered multiple bilateral pneumothoraxes but didn’t respond to tube thoracostomy and chemotherapy management. The patient died due to respiratory failure raised from complications of lung involvement as a multisystem LCH, 29 days later.
Pediatricians should pay much more attention to the cutaneous lesions in the neonatal period especially if there is any risk factor for presenting LCH such as IVF. The lesions should be monitored closely owing to a high correlation between skin lesions and MS LCH.