Letterer–Siwe disease

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Letterer–Siwe disease
Classification and external resources
ICD-10 C96.0
ICD-9 202.5
ICD-O 9722/3
OMIM 246400
DiseasesDB 5906

Letterer–Siwe disease is an old name for Langerhans cell histiocytosis (LCH), from a time when LCH was thought to be several different diseases; Letterer–Siwe disease, Hand-Schuller-Christian disease, Eosinophilic granuloma and Hashimoto-Pritzker disease. Later they were all put together under the name Histiocytosis X. The X was found to be the Langerhans cell, a dendritic white blood cell. LCH is not believed to be genetic, and even though there have been a few reports of more than one person affected by LCH within the same family, this is not considered to be the answer to the mystery of Langerhans cell histiocytosis. Still to this day, the cause is unknown, after researchers have worked very hard on trying to figure it out for decades.

The old name, Letterer-Siwe disease, is derived from the names of Erich Letterer and Sture Siwe.

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