Nodules in the newborn- 3 cases of congenital self-healing histiocytosis

Langerhans Cell Histiocytosis (LCH) is an uncommon and unusual clonal disorder with diverse clinical presentations in children, adolescents, and adults.
Congenital self-healing histocytosis (CSHR, or Hashimoto-Pritzker disease) is a rare, spontaneously regressing variant of LCH. It is characteristically seen in well infants, with isolated cutaneous involvement.
CSHR often presents at birth or in the newborn period. It can present with solitary or more commonly, multiple cutaneous nodules anywhere on the body. They can centrally ulcerate either before or shortly after birth, often heal within a few months, and can sometimes leaving an atrophic scar.
CSHR is diagnosed by skin biopsy. Histology shows a proliferation of mononuclear cells resembling Langerhans cells, and positive immunohistochemical staining of infiltrative cells in the dermis with CDla and S-100
No treatment is required for CSHR. However, patients require further evaluation and monitoring, due to the possibility of recurrence or progression-a review of 126 reported cases of CSHR found a recurrence rate of 10% (all within the first year of life) and a mortality rate of 3%.(1) Therefore, it is advised that the patient has oncological and paediatric follow-up with appropriate investigations to rule out liver, splenic or bone marrow involvement.
In addition to the epidemiology and pathophysiology of CSHR, I shall discuss the differential diagnosis of two neonates who present with nodular lesions. Illustrative cases will be used.
1. 1. Longaker MA, Frieden IJ, LeBoit PE, Sherertz EF. Congenital "self-healing" Langerhans cell histiocytosis: the need for long-term follow-up. Journal of the American Academy of Dermatology. 1994;31(5 Pt 2):910-6.

Dr. Charlotte Wyatt

Sydney Children's Hospital

Charlotte is currently working as a registrar in the Sydney Children's Network. She is originally from the United Kingdom, having studied Graduate Medicine at the University of Southampton. Prior to that she read Music at the University of Oxford. Charlotte worked as an honorary registrar in Dermatology at the Prince Alexandra Hospital, Brisbane. She has an interest in paediatric dermatology and medical student teaching.