Chemotherapy is indicated for patients with multisystem involvement. Patients who do not respond to even aggressive chemotherapy may consider reduced-intensity hematopoietic stem cell transplantation, experimental chemotherapy, or immunomodulatory therapy specific to any identified mutation. Patients with BRAFV600E mutations, for example, may be candidates for BRAF inhibitors such as vemurafenib. Early consultation with a dermatologist in patients with skin manifestations is key, as early diagnosis and treatment can be life-saving to patients with this often terminal disease.
Letterer–Siwe disease
Fox–Fordyce disease
Brill–Zinsser disease
Paget–Schroetter disease
Laurence–Moon syndrome
Kapur–Toriello syndrome
Degos disease
Landau–Kleffner syndrome