scleromyxedema
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.
MONDO:0018432
lichen myxedematosus
is_a
Arndt-Gottron disease
exact
Scleromyxedema
exact
generalised lichenoid papular eruption
exact
generalised papular and sclerodermoid
related
generalised papular and sclerodermoid lichen myxedematosus
exact
generalized lichenoid papular eruption
exact
generalized papular and sclerodermoid
related
generalized papular and sclerodermoid lichen myxedematosus
exact
lichen myxedematosus
related
mucinosis, papular
related
myxedematosus, lichen
related
papular mucinosis
related
scleromyxoedema
exact