Scleromyxedema

Scleromyxedema

Definition

Scleromyxedema is a rare, severe skin disorder. Signs and symptoms include abnormal accumulation of mucin in the skin (mucinosis), causing papular and sclerodermoid bumps; increased production of fibroblasts (connective tissue cells) in the absence of a thyroid disorder; and monoclonal gammopathy (abnormal proteins in the blood). It often involves internal organs and may affect various body systems. The cause of scleromyxedema is not known. There is no standard treatment. Management may involve the use of intravenous immunoglobulin (IVIG), plasmapheresis, thalidomide and corticoids, or more aggressive interventions, such as autologous bone marrow transplantation.

Also known as Arndt-Gottron disease, generalised lichenoid papular eruption, generalised papular and sclerodermoid lichen myxedematosus, generalized lichenoid papular eruption, generalized papular and sclerodermoid lichen myxedematosus, Scleromyxedema, scleromyxoedema — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Integumental system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Skin of body Disease Has Primary Anatomic Site NCIT · CC BY 4.0