Histiocytoses – Congenital Self Healing Reticulohistiocytosis

Congenital Self Healing Reticulohistiocytosis (CSHR), also called Hashimoto-Pritzker Disease, is a very rare X-Type Histiocytosis that I would only rarely encounter in both my The Woodlands dermatology and Conroe dermatology offices.  CSHR usually occurs at birth and can occur as either a solitary or generalized variant.  Clinically, the lesions in CSHR are red/brown nodules that can become very large and ulcerate.  In most cases, the lesions involute spontaneously over several months.  Systemic involvement is absent in CSHR unlike in Langerhans Cell Histiocytosis.  It is important to evaluate for internal involvement to rule out the more serious Langerhans Cell Histiocytosis.