Congenital Langerhans cell histiocytosis with skin and lung involvement: spontaneous regression

Indian J Pediatr. 2010 Jul;77(7):811-2. doi: 10.1007/s12098-010-0104-3. Epub 2010 Jun 29.

Abstract

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare type of Langerhans Cell Histiocytosis, presenting at birth or during the neonatal period with eruption of multiple, discrete red-brown papules or nodules that undergo spontaneous regression. Systemic signs are generally absent. We describe a 4-month-old infant presenting with reddish brown nodular cutaneous lesions since birth with a past history suggestive of pulmonary involvement. Skin biopsy showed a dermal infiltrate of pleomorphic histiocytes; which were CD1a and S-100 positive, consistent with the diagnosis of CSHLCH. Both pulmonary and cutaneous lesions showed spontaneous resolution.

Publication types

  • Case Reports

MeSH terms

  • Histiocytosis, Langerhans-Cell / complications
  • Histiocytosis, Langerhans-Cell / congenital*
  • Histiocytosis, Langerhans-Cell / pathology
  • Humans
  • Hypopigmentation / etiology
  • Infant
  • Male
  • Remission, Spontaneous
  • Respiration Disorders / etiology*
  • Skin Diseases / etiology*
  • Skin Diseases / pathology