Congenital self-healing reticulohistiocytosis (CSHR) is a rare variant of Langerhans cell histiocytosis (LCH). The incidence of CSHR is around 1-5 cases per million, although this may be underreported due to the high rate of spontaneous resolution. Males and females are equally affected, and there seems to be a higher prevalence among White neonates. As a variant of LCH, it is a clonal proliferative disorder; however, the etiology remains unknown.

While the vast majority of patients have disease limited to the skin, rare pulmonary or ocular involvement has been described.

CSHR is usually a benign, self-limited disease that does not require any specific treatment, as the skin and pulmonary / eye lesions spontaneously involute; however, relapses in skin lesions or lesions at extracutaneous sites (lung or eye) have been reported up to 4 years after resolution of the initial lesions. Because it is difficult to differentiate this condition from classic LCH, a skin biopsy, systemic evaluation (laboratory testing / imaging), and close follow-up should be performed. Overall, the prognosis is good.