- Children <2yo (variable)
- Skin 80% of cases, usually initial sign
- Red-brown papules on ears, scalp, intertriginous
- Resembles hemorrhagic seborrheic dermatitis, involves skin folds (~candida)
- Often systemic involvement, acutely ill
- Lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, fever, pulmonary and neurologic symptoms
- Bone lesions rarer than other LCH, mastoid possible in all forms
- Mortality >50%, worse prognosis if poor response to initial therapy
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