National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Scleromyxedema


Other Names:
Arndt-Gottron disease; Generalized lichenoid papular eruption; Generalized papular and sclerodermoid ; Arndt-Gottron disease; Generalized lichenoid papular eruption; Generalized papular and sclerodermoid ; Generalized papular and sclerodermoid lichen myxedematosus See More
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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. 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.[2]
Last updated: 9/29/2016
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 and the range of motion of the face, fingers, and extremities is decreased.

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
  • Breathing difficulty caused by restrictive and obstructive pulmonary dysfunction
  • Heart abnormalities.
Last updated: 9/29/2016
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: 3/13/2017
To our knowledge, there is currently no evidence that scleromyxedema is inherited. We are not aware of any reports in the medical literature describing scleromyxedema affecting more than one member of a family.
Last updated: 3/13/2017
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 and usually is used first; this type of treatment can have relatively long-term effects and few side effects. Because of this, it is currently considered by many the best treatment option. Plasmapheresis is effective as a short-term treatment but leads to relapses (recurrence of symptoms).[2] For those patients who cannot receive IVIG, thalidomide and systemic glucocorticoids may be used. When the patients with severe disease do not have a good response, other interventions are required, such as autologous bone marrow transplantation, melphalan,or bortezomib with dexamethasone.[1] 
Last updated: 9/29/2016

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Scleromyxedema Survivors
    C/O Phyllis Kay Phillips
    1704 Lower Millstone Lane
    Salisbury, MD 21801
    Telephone: 410-742-7843

Social Networking Websites

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Scleromyxedema. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • My husband was 43 years old when he died from complications associated with scleromyxedema. We have 2 daughters and one son. Is it hereditary, and should they be tested for the disease? See answer

  • What causes scleromyxedema and how is it treated? See answer


  1. Rongioletti F. Scleromyxedema. UpToDate. Waltham, MA: UpToDate; 2015;
  2. 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.
  3. Liotta E.A. Lichen Myxedematosus. Medscape Reference. 2016; http://www.emedicine.com/DERM/topic231.htm.
  4. Eugene Tan. Lichen myxoedematosus. DermNet NZ. 2008; http://dermnetnz.org/immune/scleromyxoedema.html.