Catalog

Hashimoto-Pritzker syndrome

No MMS code

Link to WHO Definition

Hashimoto-Pritzker syndrome – http://id.who.int/icd/entity/1011705244

Hashimoto-Pritzker syndrome, also known as congenital self-healing reticulo-histiocytosis or Hashimoto-Pritzker histiocytosis (HPH) is a variant of Langerhans cell histiocytosis. Classic signs and symptoms include congenital or early development of red-brown skin lesions in the form of painless papules, nodules or plaques with spontaneous regression in 2-3 months, and increase of a type of immune cells known as Langerhans cell histiocytes. An excess of immature Langerhans cells usually forms tumors called granulomas. Most patients have multiple lesions, but in about 25% of cases there is only one lesion. Development of lesions in adulthood, recurrence of the disease, as well as lung and eye involvement, are very rare.