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

• Localised Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms
• Localized Lichen Myxoedematosus with Monoclonal Gammopathy and/or Systemic Symptoms

What is Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms? (Definition/Background Information)

• Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms is an atypical form of lichen myxedematosus, which is a rare skin abnormality characterised by mucin deposition in the skin
• In Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms, the features of both the localized form and generalized form of lichen myxedematosus are noted. Thus, systemic symptoms are also present in addition to cutaneous manifestations
• Monoclonal gammopathy indicates an abnormal level of certain protein complexes in blood, which can increase one’s risk for blood and bone marrow disorders
• Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms is a very uncommon skin condition that presents multiple skin-colored papules anywhere on the body. Additionally, systemic symptoms involving several parts of the body including the lungs, muscles and joints, etc. may be present
• The exact cause of development of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms is currently unknown. No predisposing factors have also been currently established for this condition
• The condition is diagnosed by a skin biopsy. Following the diagnosis of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms, the treatment is undertaken via administration of corticosteroids, intravenous immunoglobulin, and a bone marrow transplant, if needed
• The prognosis of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms depends upon the severity of the condition. In many cases, the condition does not respond well to therapy and the prognosis can be unpredictable

Who gets Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms? (Age and Sex Distribution)

• Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms is an extremely rare skin condition that is usually diagnosed in older 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 Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms? (Predisposing Factors)

• No specific risk factors are identified for Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms

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 Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms? (Etiology)

The exact cause of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms (DPLM) formation is generally unknown.

• It may be due to an abnormal immune function that may involve the skin and/or other parts of the body (systemic condition)
• 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 the condition through direct contact or from being in close proximity to an individual having the condition.

What are the Signs and Symptoms of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms?

The signs and symptoms of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms may include:

• Presence of numerous, small round papules on skin that are firm and flesh-colored
• 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 can be present anywhere on the body; on the head and neck region, limbs (arms and legs), and chest and back
• Thickening of skin may be observed

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. Nevertheless, systemic symptoms may or may not be observed.

How is Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms Diagnosed?

Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms 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
• Blood and urine tests; protein electrophoresis
• Thyroid function test
• Antinuclear antibody (ANA) blood test
• 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 Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms?

The complications of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms 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)
• Muscle weaknesses due to myopathy
• Numbness of toes and fingers due to Raynaud’s phenomenon
• Bone marrow dysfunction

How is Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms Treated?

Currently, there is no specific treatment for Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms. It is also generally difficult to treat this progressive condition, especially if systemic symptoms are observed. The following treatment measures may be considered:

• Use of topical steroids and oral retinoid
• 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
• Dermabrasion
• CO2 laser therapy
• Intralesional steroid injections and intralesional hyaluronidase injections
• Use of systemic steroids
• Autologous bone marrow transplantation, for severe disease
• Administration of chemotherapy medications
• Symptomatic and supportive therapy, if needed

How can Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms be Prevented?

Current medical research has not established a method of preventing the occurrence of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms.

What is the Prognosis of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms? (Outcomes/Resolutions)

• The prognosis of Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms 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 Localized Lichen Myxedematosus with Monoclonal Gammopathy and/or Systemic Symptoms:

• 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