What are the other Names for this Condition? (Also known as/Synonyms)

• Arndt-Gottron Syndrome
• Generalized Lichen Myxedematosus
• Generalized Papular and Sclerodermoid Lichen Myxedematosus

What is Scleromyxedema? (Definition/Background Information)

• Scleromyxedema is the generalized form of lichen myxedematosus, which is a rare skin abnormality characterised by mucin deposition in the skin
• Scleromyxedema, also known as Arndt-Gottron Disease, is a severe condition that presents tiny, well-formed, skin-colored papules all over the body, particularly on the upper and lower limbs and trunk region
• Apart from the skin, Scleromyxedema may involve several body systems such as the bones and muscles, central nervous system, digestive system, and respiratory system, resulting in complications that include arthritis, swallowing difficulties, and pulmonary dysfunction
• The exact cause of development of Scleromyxedema is currently unknown. Occasionally, the condition is seen in association with blood malignancies such as leukemia and lymphoma
• The condition is diagnosed by a skin biopsy. Following the diagnosis of Scleromyxedema, the treatment is undertaken via administration of corticosteroids, intravenous immunoglobulin, and a bone marrow transplant, if needed
• The prognosis of Scleromyxedema depends upon the severity of the condition. In many cases, the condition does not respond well to therapy and its prognosis is unpredictable, since Scleromyxedema has the potential to involve several vital body organs

Who gets Scleromyxedema? (Age and Sex Distribution)

• Scleromyxedema is a rare skin condition with about 150 cases recorded in the medical literature
• The condition is usually diagnosed in young and middle-aged adults in the 30-50 years’ age group
• Both males and females are affected
• All racial and ethnic groups are affected and no predilection is reported

What are the Risk Factors for Scleromyxedema? (Predisposing Factors)

Currently, there are no specific risk factors identified for Scleromyxedema.

• Occasionally, a few cases have been associated with blood and bone marrow conditions such as leukemia, lymphoma, and multiple myeloma
• Presently, a family history of the condition has not been reported

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Scleromyxedema? (Etiology)

• The exact cause of Scleromyxedema formation is generally unknown
• It is known to be associated with monoclonal gammopathy, in a majority of cases. Monoclonal gammopathy is a disorder wherein there is an increase of immunoglobulin, a type of protein that helps with immunity, in the body

It is a non-contagious condition that cannot spread from one individual to another. Hence, one cannot get Scleromyxedema through direct contact, or by being in close proximity to an individual with the condition.

What are the Signs and Symptoms of Scleromyxedema?

The signs and symptoms of Scleromyxedema may include:

• Presence of numerous, small round papules on skin that are between 3-5 mm in size. The tiny bumps are arranged close to each other in a linear pattern
• The skin eruptions are waxy, firm and usually flesh-colored, but sometimes they may be reddish-brown too
• A papule is an area of abnormal skin tissue that is less than 1 centimeter around. Usually a papule has distinct borders, and it can appear in a variety of shapes
• The papules are dome-shaped and densely present anywhere on the body, notably on the arms and legs (including elbows and knees), thighs, chest and back, and the head and neck region (including the face)
• The mucus membranes and scalp are generally spared
• Over time, a thickening and stiffening of skin tissue may be noted
• Extensive skin lesions at a joint may decrease the range of movement of the involved joint
• Lesions on the face may cause difficulty in closing or opening one’s mouth 

Besides the skin, the condition may involve several parts of the body such as the upper airways, pharynx, eyes, voice box, gastrointestinal tract, and heart, leading to severe symptoms.

How is Scleromyxedema Diagnosed?

Scleromyxedema is diagnosed through the following tools:

• Complete physical examination with evaluation of medical history
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood and urine tests; protein electrophoresis
• Quantitative analysis of immunoglobulins and an evaluation of their diversity (polyclonal or monoclonal)
• Thyroid function test
• Antinuclear antibody (ANA) blood test
• Tests and procedures to detect an underlying condition, if any present
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Scleromyxedema?

Several body systems are affected by Scleromyxedema and the complications observed may include:

• Scratching or itching of the lesions may lead to bleeding and ulceration. This can cause secondary bacterial or fungal infections to develop
• Severe emotional stress due to cosmetic concerns
• Loss of vision or impaired vision
• Abnormal lung function causing breathing difficulties
• Swallowing difficulties due to lack of esophageal muscle contraction
• Hoarse voice
• Arthritis involving several body joints (polyarthritis)
• Carpal tunnel syndrome
• Muscle weaknesses due to myopathy
• Numbness of toes and fingers due to Raynaud’s phenomenon
• Central nervous system involvement causing brain damage
• Kidney failure
• Heart abnormalities
• Bone marrow dysfunction

How is Scleromyxedema Treated?

Currently, there is no specific treatment for Scleromyxedema. It is also generally difficult to treat this progressive condition. Long-term and often aggressive therapy is needed to address the severe symptoms and multi-system complications that arise.

The following treatment measures for Scleromyxedema may be considered:

• Administration of intravenous immunoglobulin (IVIG) is reportedly an effective treatment option
• Plasmapheresis; the removal of exchange of certain components of blood
• Light therapy; psoralen and ultraviolet A (PUVA) therapy
• Use of systemic steroids
• Autologous bone marrow transplantation, for severe disease
• Administration of chemotherapy medications
• Symptomatic and supportive therapy

How can Scleromyxedema be Prevented?

Current medical research has not established a method of preventing the development of Scleromyxedema.

What is the Prognosis of Scleromyxedema? (Outcomes/Resolutions)

• The prognosis of Scleromyxedema is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis of Scleromyxedema may be only assessed on a case-by-case basis

Additional and Relevant Useful Information for Scleromyxedema:

• It is advised to not pick or scratch the skin lesions
• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition