FAMILIAL LICHEN AMYLOIDOSIS (TYPE IX)

Familial Lichen Amyloidosis (Type IX) is a skin disorder in which faulty protein clumps known as amyloids form in the wave-like extensions between the upper two layers of skin. Skin patches with atypical texture or color are the predominant symptom of PLCA.

These patches distinguish three types of amyloidosis: macular, lichen, and nodular. Mutations in the IL31RA or OSMR genes can result in familial lichen amyloidosis (type IX). Moreover, the type 1 conditions are caused by mutations in the IL31RA gene. Additionally, macular and lichen are expected to be linked to diseases with comparable disease processes, and mutations in either gene are believed to be involved.

SYMPTOMS

Signs of familial lichen amyloidosis (type IX) may not appear until the disease has progressed. When symptoms arise, they are determined by which organs are impacted.

The following are possible symptoms:

  • Extreme exhaustion and weakness
  • Inflamed legs and ankles
  • Breathing problems
  • Tingling, numbness, or discomfort in the feet or hand, particularly in the wrist
  • Sudden weight loss
  • Trouble swallowing
  • Skin changes
  • Abnormal heartbeat
  • Enlarged tongue

DIAGNOSIS

Since the symptoms of this disorder might be mistaken for those of more prevalent diseases, it is frequently neglected. However, early detection can aid in the prevention of further complications. Because therapy varies widely based on your exact condition, it’s critical to have an accurate diagnosis.

Some of the tests required are the following:

  • Laboratory tests. It’s possible that your urine and blood will be tested for abnormal proteins that show amyloidosis.
  • Biopsy. It’s possible to collect a tissue sample and look for symptoms of amyloidosis. The biopsy could come from the fat beneath your skin on your stomach, bone marrow, or a damaged organ.
  • Imaging tests. The severity of your disease can be determined using images of the organs damaged by amyloidosis. Nuclear imaging, MRI, and echocardiogram are some of the tests that may be performed.

TREATMENT

Familial lichen amyloidosis (type IX) has no treatment. Therapy, on the other hand, can help control symptoms while also limiting amyloid protein formation. If another disease, such as rheumatoid arthritis, caused the amyloidosis, addressing the underlying ailment may be beneficial.

Medication

  • Targeted therapies
  • Heart medications
  • Chemotherapy

Surgical and other interventions

  • Organ transplant
  • Dialysis
  • Autologous blood stem cell transplant

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