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Genetic and Rare Diseases Information Center (GARD)

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Scleromyxedema


Other Names for this Disease

  • Arndt-Gottron disease
  • Generalized lichenoid papular eruption
  • Generalized papular and sclerodermoid
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

What causes scleromyxedema and how is it treated?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is 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.[1][2] The cause of scleromyxedema is not known. Management may involve the use of intravenous immunoglobulin (IVIG) and/or plasmapheresis, but no standard treatment exists.[2]
Last updated: 4/18/2014

What are the signs and symptoms of scleromyxedema?

Scleromyxedema usually affects people between the ages of 30 and 50.[3] Skin symptoms usually include a generalized papular eruption with sclerosis (hardening of tissue). 

The papules may be dome shaped, firm, skin colored, or red, and approximately 3 mm in diameter.  Extensive areas of the skin may be involved. The face, knees, and elbows are often affected.

Scleromyxedema can involve areas of the body other than the skin, including the pharynx and the upper airway. Other symptoms that can be caused by scleromyxedema include:
  • Esophageal aperistalsis (absence of muscular contractions that help us swallow)  
  • Hoarseness  
  • Inflammatory polyarthritis (simultaneous inflammation of several joints) 
  • Proximal myopathy (various conditions or diseases of the muscular tissues) 
  • Neurologic dysfunction 
  • Eye abnormalities
Last updated: 1/6/2009

What causes scleromyxedema?

The underlying cause of scleromyxedema remains unknown.[2][1] In a few cases, it has been reported in association with cancers of the bone marrow such as myeloma, lymphoma and leukemia.[4]
Last updated: 4/18/2014

How might scleromyxedema be treated?

There is no standard treatment for scleromyxedema. The severe course of the disease requires very aggressive treatment, and long-term maintenance therapy is usually necessary. According to the literature, the use of intravenous immunoglobilin (IVIG) may be successful; this type of treatment can have relatively long-term effects and few side effects. Because of this, it is currently considered the best treatment option. Plasmapheresis is effective as a short-term treatment and leads to relapses (recurrence of symptoms).[2]
Last updated: 4/18/2014

References
  • Franco Rongioletti. Scleromyxedema. UpToDate. Waltham, MA: UpToDate; April, 2014; Accessed 4/18/2014.
  • Koronowska SK, Osmola-Mankowska A, Jakubowicz O, Zaba R. Scleromyxedema: a rare disorder and its treatment difficulties. Postepy Dermatol Alergol. April, 2013; 30(2):122-126. Accessed 4/18/2014.
  • Liotta E.A. Lichen Myxedematosus. eMedicine. 2006; http://www.emedicine.com/DERM/topic231.htm. Accessed 1/5/2009.
  • Eugene Tan. Lichen myxoedematosus. DermNet NZ. 2008; http://dermnetnz.org/immune/scleromyxoedema.html. Accessed 4/18/2014.
Other Names for this Disease
  • Arndt-Gottron disease
  • Generalized lichenoid papular eruption
  • Generalized papular and sclerodermoid
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.