Congenital self-healing reticulo-histiocytosis

This neoplasm is not reportable.
Name
Congenital self-healing reticulo-histiocytosis
Not Reportable
This neoplasm is not reportable
Alternate Names
Congenital reticulohistiocytosis
Congenital self-healing Langerhans cell histiocytosis
CSHRH
Hashimoto-Pritzker disease
Definition
Rare condition, about 5% of all langerhan cell histiocytosis cases, which present usually at birth or in the neonatal period, with solitary, localized to generalized papules, vesicles, or nodules on the trunk, head, palms and soles.

Within several weeks, lesions tend to involute spontaneously leaving behind hypo- or hyperpigmented macules or patches.
Abstractor Notes
Patients with this condition should be monitored carefully for relapses. Relapses often involve the bone and may progress to Letterer-Siwe disease, which is a disseminated form of Langerhans cell histiocytosis (9751/3).