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

• Atypical form of Lichen Myxedematosus
• Atypical form of Papular Mucinosis
• Intermediate Lichen Myxedematosus

What is Atypical Lichen Myxedematosus? (Definition/Background Information)

• Atypical Lichen Myxedematosus is a skin condition that comprises of subtypes that may exhibit features from both the generalized form and localized forms of lichen myxedematosus, which is a rare skin abnormality characterised by mucin deposition in the skin
• The generalized form is often severe and presents systemic signs and symptoms, while localized forms are usually milder and are characterised by skin manifestations only, in a vast majority of cases. The exact cause of development of Atypical Lichen Myxedematosus is currently unknown
• Atypical Lichen Myxedematosus constitute the following four subtypes:
  • Scleromyxedema without monoclonal gammopathy
  • Localized lichen myxedematosus with monoclonal gammopathy and/or systemic symptoms
  • Localized lichen myxedematosus with mixed features of different subtypes
  • Not otherwise specified (NOS); includes cases that may not be grouped into any of the above subtypes
• Atypical Lichen Myxedematosus is a highly uncommon condition that presents tiny skin-colored papules all over the body. Apart from the skin, some subtypes may involve several body systems such as the bones and muscles, central nervous system, digestive system, and respiratory system, resulting in severe complications
• The condition is diagnosed by a skin biopsy. Following the diagnosis of Atypical Lichen Myxedematosus, the treatment is undertaken for skin symptoms and systemic symptoms, if present. The treatment may include light therapy, use of topical creams, dermabrasion, administration of corticosteroids, and intravenous immunoglobulin
• The prognosis of Atypical Lichen Myxedematosus depends upon the severity of the condition. In some subtypes, the condition does not respond well to therapy and the prognosis may be unpredictable, since it can potentially involve several vital organs of the body

Who gets Atypical Lichen Myxedematosus? (Age and Sex Distribution)

• Atypical Lichen Myxedematosus is a very rare skin condition that may be diagnosed in adults and children, depending on the specific subtype of the condition observed
• Both males and females are affected
• All racial and ethnic groups are affected and no predilection is reported

What are the Risk Factors for Atypical Lichen Myxedematosus? (Predisposing Factors)

• Currently, there are no specific risk factors identified for Atypical Lichen Myxedematosus

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 Atypical Lichen Myxedematosus? (Etiology)

• The exact cause of Atypical Lichen Myxedematosus formation is generally unknown
• 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 by being in close proximity to an individual having the condition

What are the Signs and Symptoms of Atypical Lichen Myxedematosus?

The signs and symptoms of Atypical Lichen Myxedematosus depend on the specific subtype of the condition. In general, the signs and 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 across. 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, larynx (voice box), gastrointestinal tract, and heart, leading to severe symptoms. Nevertheless, systemic symptoms may or may not be observed and is dependent upon the specific subtype of Atypical Lichen Myxedematosus.

How is Atypical Lichen Myxedematosus Diagnosed?

Atypical Lichen Myxedematosus 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 (CBC) test
• Blood and urine tests; protein electrophoresis
• 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 Atypical Lichen Myxedematosus?

Several body systems may be affected by Atypical Lichen Myxedematosus based upon the characteristic subtype noted. The localized myxedematosus with monoclonal gammopathy and/or systemic symptoms and the localized lichen myxedematosus with mixed features of different subtypes may or may not present any systemic symptoms.

Some of 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
• Central nervous system involvement causing brain damage
• Kidney failure
• Heart abnormalities

How is Atypical Lichen Myxedematosus Treated?

Currently, there is no specific treatment for Atypical Lichen Myxedematosus. It is also generally difficult to treat this progressive condition. In some forms, a spontaneous resolution of the condition may be noted. Otherwise, long-term and often aggressive therapy is needed to address the severe symptoms and multi-system complications that arise.

The following treatment measures may be recommended in treating Atypical Lichen Myxedematosus depending on the combination of underlying signs and symptoms noted.

In case of skin-related symptoms:

• Use of topical steroids and oral retinoid
• Psoralen and ultraviolet A (PUVA) therapy
• Dermabrasion
• CO2 laser therapy
• Intralesional steroid injections and intralesional hyaluronidase injections
• Newer treatment methods, such as topical tacrolimus creams

In case of systemic symptoms that affect the whole body:

• Administration of intravenous immunoglobulin (IVIG)
• Plasmapheresis; the removal of certain components of blood
• Use of systemic steroids
• Autologous bone marrow transplantation, for severe disease
• Administration of chemotherapy medications
• Symptomatic and supportive therapy

How can Atypical Lichen Myxedematosus be Prevented?

Current medical research has not established a method of preventing the occurrence of Atypical Lichen Myxedematosus.

What is the Prognosis of Atypical Lichen Myxedematosus? (Outcomes/Resolutions)

• The prognosis of Atypical Lichen Myxedematosus is dependent upon the specific subtype and the severity of the signs and symptoms and associated complications, if any present
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications. Thus, individuals with ‘skin-alone’ symptoms have better prognoses than those with symptoms that involve several or entire body systems
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Atypical Lichen Myxedematosus:

• 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