Congenital self-healing histiocytosis (Hashimoto-Pritzker). A study of eleven cases.

Abstract

Congenital self-healing Langerhans cell histiocytosis is a rare disorder in the neonatal period. It manifests itself with papules, vesicles or nodules and does not affect other organs besides the skin. The cutaneous lesions tend to regress spontaneously within weeks or months. This report includes the clinical features and laboratoy findings of eleven infants with congenital self-healing Langerhans cell histiocytosis, which were observed in Buenos Aires in the period between January, 1989 and May, 1998. In these eleven cases are evaluated the sex, age of onset, type of cutaneous lesions, color, presence of erosion, affected sites and time of healing. The Authors believe that the disorder is more frequent than reported, because of its rate of rapid spontaneous resolution. They propose that in all patients with this disorder a systemic evaluation must be done to exclude any possible extracutaneous involvement. The Authors also suggest a long term follow up to put in evidence relapse or progression of the disease.