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

• Scleromyxoedema without Monoclonal Gammopathy

What is Scleromyxedema without Monoclonal Gammopathy? (Definition/Background Information)

• Scleromyxedema without Monoclonal Gammopathy is an atypical form of lichen myxedematosus, which is a rare skin abnormality characterized by mucin deposition in the skin
• Scleromyxedema without Monoclonal Gammopathy is highly uncommon and severe condition that presents tiny, well-formed, skin-colored papules all over the body, particularly on the face, upper and lower limbs, and trunk region
• The exact cause of development of Scleromyxedema without Monoclonal Gammopathy is currently unknown. Monoclonal gammopathy indicates an abnormal level of certain protein complexes in blood, which can increase one’s risk for blood and bone marrow disorders. In over 80% of the individuals with scleromyxedema, monoclonal gammopathy is present
• Apart from the skin, Scleromyxedema without Monoclonal Gammopathy 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 condition is diagnosed by a skin biopsy, with blood tests to determine the presence or absence of monoclonal gammopathy. Following the diagnosis of Scleromyxedema without Monoclonal Gammopathy, the treatment is undertaken via administration of corticosteroids, intravenous immunoglobulin, and bone marrow transplantation, if needed
• The prognosis of Scleromyxedema without Monoclonal Gammopathy depends upon the severity of the condition. In many cases, the condition does not respond well to therapy and its prognosis is unpredictable, since it has the potential to involve several vital organs of the body

Who gets Scleromyxedema without Monoclonal Gammopathy? (Age and Sex Distribution)

• Scleromyxedema without Monoclonal Gammopathy is a very rare skin condition that may be diagnosed in adults
• 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 without Monoclonal Gammopathy? (Predisposing Factors)

• Currently, there are no specific risk factors identified for Scleromyxedema without Monoclonal Gammopathy

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 without Monoclonal Gammopathy? (Etiology)

• The exact cause of Scleromyxedema without Monoclonal Gammopathy formation is generally unknown
• In a majority of cases, scleromyxedema is known to be associated with monoclonal gammopathy. Monoclonal gammopathy is a disorder wherein there is an increase of immunoglobulins, 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. This means that one cannot get Scleromyxedema through direct contact or from being in close proximity to an individual having the condition.

What are the Signs and Symptoms of Scleromyxedema without Monoclonal Gammopathy?

The signs and symptoms of Scleromyxedema without Monoclonal Gammopathy may include:

• Presence of numerous, small round papules on skin that are between 1-3 mm in size. The tiny bumps are arranged close to each other in a linear or grouped pattern
• The skin eruptions are waxy, firm and usually flesh-colored, but sometimes they may be reddish-brown. Unlike other subtypes of papular mucinosis, itching is prominently noted in this form
• A papule is an area of abnormal skin tissue that is less than 1 centimeter across. 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 face, chest and back, and arms and legs
• When lesions are present on the face, it may give a lion-like appearance
• 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. However, enlarged liver and spleen (hepatomegaly and splenomegaly respectively) are not noted.

How is Scleromyxedema without Monoclonal Gammopathy Diagnosed?

Scleromyxedema without Monoclonal Gammopathy may be 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
• Complete blood count; this is usually normal
• Blood and urine tests; both serum immunoelectrophoresis and urine electrophoresis are usually normal
• 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 without Monoclonal Gammopathy?

Several body systems are affected by Scleromyxedema without Monoclonal Gammopathy 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 infiltration by abnormal mucin compounds
• Hoarse voice
• Arthritis involving several body joints (polyarthritis)
• Carpal tunnel syndrome
• Muscle weaknesses due to myopathy
• Central nervous system involvement causing brain damage
• Kidney failure
• Heart abnormalities

How is Scleromyxedema without Monoclonal Gammopathy Treated?

Currently, there is no specific treatment for Scleromyxedema without Monoclonal Gammopathy. 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.

Depending on the combination of underlying signs and symptom, the following treatment measures for Scleromyxedema without Monoclonal Gammopathy may be considered:

• Administration of intravenous immunoglobulin (IVIG)
• Plasmapheresis; the removal  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 without Monoclonal Gammopathy be Prevented?

Current medical research has not established a method of preventing the occurrence of Scleromyxedema without Monoclonal Gammopathy.

What is the Prognosis of Scleromyxedema without Monoclonal Gammopathy? (Outcomes/Resolutions)

• The prognosis of Scleromyxedema without Monoclonal Gammopathy 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 may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Scleromyxedema without Monoclonal Gammopathy:

• Do 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