The disease is often rapidly fatal, with a five-year survival rate of 50%. Poor prognostic indicators include disseminated disease, age < 2 years, and the development of thrombocytopenia. The involvement of risk organs, which include the liver, spleen, and organs of the hematopoietic system, also suggests a worse prognosis and significantly higher mortality rate. The most significant prognostic factor is a response to initial (6-12 weeks) therapy with vinblastine and steroids. This factor outweighs even age as a prognostic indicator. For infants, LSD is nearly always a fatal illness. Death may occur in-utero or within a few weeks of birth.
Letterer–Siwe disease
Fox–Fordyce disease
Brill–Zinsser disease
Paget–Schroetter disease
Laurence–Moon syndrome
Kapur–Toriello syndrome
Degos disease
Landau–Kleffner syndrome