Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature

Sultan Qaboos Univ Med J. 2017 May;17(2):e225-e228. doi: 10.18295/squmj.2016.17.02.016. Epub 2017 Jun 20.

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. There was a delayed response to combined chemotherapy with no major side-effects.

Keywords: Anemia; Case Report; India; Infant; Langerhans Cell Histiocytosis; Seborrheic Dermatitis; Tachypnea.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Antineoplastic Agents, Phytogenic / therapeutic use
  • Biopsy
  • Delayed Diagnosis
  • Disease Progression
  • Drug Therapy, Combination
  • Exanthema / drug therapy*
  • Exanthema / pathology
  • Histiocytosis, Langerhans-Cell / diagnosis
  • Histiocytosis, Langerhans-Cell / drug therapy*
  • Humans
  • India
  • Infant
  • Langerhans Cells / pathology
  • Lung / diagnostic imaging
  • Male
  • Prednisone / therapeutic use
  • Skin / pathology
  • Vinblastine / therapeutic use

Substances

  • Anti-Inflammatory Agents
  • Antineoplastic Agents, Phytogenic
  • Vinblastine
  • Prednisone