Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations

Ital J Pediatr. 2021 Jun 10;47(1):135. doi: 10.1186/s13052-021-01082-9.

Abstract

Background: Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement.

Case presentation: We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years.

Conclusions: Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder.

Keywords: CSHRH; Congenital self-healing reticulohistiocytosis; Hashimoto-Pritzker disease; Histiocytosis; Newborn.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Biopsy
  • Histiocytosis, Langerhans-Cell / congenital*
  • Histiocytosis, Langerhans-Cell / pathology*
  • Humans
  • Infant, Newborn
  • Male
  • Remission, Spontaneous
  • Skin / pathology

Supplementary concepts

  • Hashimoto-Pritzker syndrome