Scleromyxedema without monoclonal gammopathy

Definition

Scleromyxedema without monoclonal gammopathy is a form of atypical lichen myxedematosus (see this term), 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.

Disease data

Classification

Clinical subtype

ORPHA code

90400

OMIM code

ICD10 code

L98.5

ICD11 code

*Soruce