Scleromyxedema without monoclonal gammopathy
ORPHA:90400· ICD-10 L98.5
Definition
Scleromyxedema without monoclonal gammopathy is a form of atypical lichen myxedematosus, characterized by a generalized sclerodermoid infiltration of skin studded with multiple, firm papules of 1-3 mm in diameter involving face (leonine appearance), trunk, and limbs, without monoclonal gammopathy. The involvement of the face can be missing and pruritus may be prominent.
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adolescent, Adult, Elderly