Although LSD has been described in adult
patients, it is predominantly a condition affecting children age 2 months to
3 years. Clinical features include fever, anemia, thrombocytopenia, and the
manifestations of histiocyte proliferation, including skin disorders
(seborrheic, eczematous, pustular or nodular lesions particularly on the
scalp), lytic lesions of the bones, and splenogenic thrombocytopenia. The
lungs can be involved (nonproductive cough, dyspnea, pleural effusion,
interstitial pneumonitis, and spontaneous pneumothorax). Hypothalamic
involvement resulting in diabetes insipidus (DI). Eye protrusion may be
present. The clinical course is very variable, and spontaneous remissions
have occurred. Prognosis is generally poor. Treatment may include use of
glucocorticoids, chemotherapeutic agents, bone marrow transplantation, and
desmopressin for DI.