LETTERER-SIWE1-3 DISEASE is a rather uncommon condition which is of special interest to the otolaryngologist and the head and neck surgeon because of the clinically typical presenting manifestations involving the ears and neck.
It is characterized by marked enlargement of the lymph nodes, especially of the cervical region (Fig 1, 2), which tends to mimic a lymphoma. A seborrheic-type rash may involve the scalp (Fig 3) and extend to the ears, where a secondary otitis externa may appear. Otitis media is not uncommon. Hepatomegaly and splenomegaly usually occur. There may also be petechial hemorrhages over the body which are associated with thrombocytopenia. Rather pronounced anemia and a low-grade fever are common. There is frequently a progressive diffuse pulmonary involvement terminating in fibrosis.4 Resistance is lowered and the patient is susceptible to secondary infections.
Letterer-Siwe disease is a condition of acute reticuloendotheliosis of unknown etiology and has been classified,
FOLLETTE WJ, ENGLEWOOD , BELL JG, CLIFFS E. Letterer-Siwe Disease or Acute Histiocytosis XA Case Report. Arch Otolaryngol. 1966;84(5):563–565. doi:10.1001/archotol.1966.00760030565019