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Macular Amyloidosis - Causes, Diagnosis and Treatment

Chapter 1: What is Macular Amyloidosis?

Chapter 2: Clinical Features of Macular Amyloidosis

Chapter 3: Macular Amyloidosis Diagnosis and Treatment

Frequently Asked Questions

Key Takeaways:

  • Macular Amyloidosis is a skin condition that is characterized by hyperpigmentation patches in the skin usually on the upper back or upper arms areas.
  • The cause of hyperpigmentation is due to the accumulation of abnormal proteins on the skin known as amyloids.
  • Macular amyloidosis patches are different from other conditions that cause hyperpigmentation such as photomelanosis. They have a ripple-like design and can be mildly itchy. Dermatologists on MFine are there to help you with a diagnosis if you think you might be showing pigmentation patches of any kind or similar symptoms.
  • Macular amyloidosis can be hereditary or caused due to environmental factors. Triggers such as prolonged friction, rubbing and scratching can assert its prominence.
  • A skin biopsy is often how macular amyloidosis is diagnosed. A dermatologist may also conduct blood tests and urine tests to rule out any associated disease the patient may be suffering from.
  • Although there is no cure for macular amyloidosis, some treatments to reduce its symptoms include oral medication, ointments and creams, phototherapy and dermabrasions. It is important to consult a dermatologist to discuss the appropriate treatment option for you based on the intensity of your symptoms.  Also refer to more of our medical guides for treatment options for various other health-related conditions.

Chapter 1: What is Macular Amyloidosis?

What is Amyloidosis?

The term “Amyloid” or “Amyloidosis” is used to describe a group of rare conditions that involve the accumulation of abnormal protein substances in several organs in the body including the skin. Amyloidosis gets its name from the Latin word “Amylum” which means starch. This is because the protein deposits on skin and organs resembled that of starch when viewed under the microscope.

Amyloidosis of the Skin

Skin amyloidosis is medically known as primary localized cutaneous amyloidosis (PLCA). PLCA can be identified by the build-up of abnormal protein or amyloid clumps in the skin causing hyperpigmented patches. It is a primary condition meaning that the skin is where the signs first develop; and its localized meaning that the skin condition is restricted to a specific area of the body, for eg. arms, legs, back, neck. These pigmentation patches usually manifest in the form of a wave-like design in between the two upper layers of the skin namely, the dermis and epidermis.
The texture and colour of the skin hyper pigment differentiate primary localized cutaneous or skin amyloidosis into three forms:
1. Lichen amyloidosis
2. Nodular amyloidosis
3. Macular amyloidosis

What is Macular Amyloidosis?

Macular amyloidosis as mentioned above is one of the types of primary localised cutaneous amyloidosis where protein deposits on skin called amyloids in certain areas of the body cause hyperpigmented patches.

What makes Macular Amyloidosis different from Lichen Amyloidosis and Nodular Amyloidosis?

In the case of other primarily localized cutaneous amyloidosis types, for instance lichen amyloidosis, the pigmentation patches are reddish-brown in colour and scaly in texture. They are usually severely itchy patches along with several small bumps on thickened skin. The patches are seen generally on the shins but can also show up on other areas of the legs, on the forearms and other parts of the body.

Nodular amyloidosis manifests in the form of raised bumps or nodules on the skin that feel firm and are brown, red or pinkish in colour. These nodules, however, are not itchy. They often appear on the face, limbs, torso, and sometimes the genital area.

On the other hand, in macular amyloidosis, the patches are flat and dark brown in colour. The texture of the hyper pigment can take a uniform lace-like or rippled design in most people. Skin hyperpigmentation is common on the upper back, but can also appear on certain parts of the torso, arms and legs. Patients with macular amyloidosis complain that these patches feel mildly itchy.

Who is at risk for macular amyloidosis?

Several studies have shown that macular amyloidosis occurs more frequently in women than men - especially women who are aged between 20 and 50. It is commonly seen in people of Asian, South and Central American, and Middle Eastern descent. Research has also shown this condition can be inherited from a previous family member. If you think you may be at risk of getting macular amyloidosis, consult a dermatologist on MFine who would be able to do a thorough medical history check and assess your risk factors for this skin condition.

Chapter 2: Clinical Features of Macular Amyloidosis

Although the main macular amyloidosis causes are uncertain, this condition can erupt in patients due to hereditary factors or environmental factors. Itching, rubbing and prolonged friction to the affection skin region also contribute to its prominence.

The most noticeable sign of macular amyloidosis is the presence of small groups of hyperpigmented patches that are dusky-brown or in some patients blue-grey in colour. The pigmentation of skin is due to the accumulation of amyloids or a specific type of abnormal protein deposits on skin which give it that colour and texture. Here are some other ways macular amyloidosis presents itself in patients:

  • The patches commonly occur on the upper part of the upper back, known as the interscapular area, and are usually symmetrically distributed. Patches are also common on areas of the skin outside the joints of the upper arm.
  • Skin hyperpigmentation can be rarely seen on the face and neck areas, collarbones, chest, and armpits, although in some cases it can occur.
  • The patches in macular amyloidosis are itchy. The intensity of the itchiness can range from anywhere between mild to severe.
  • Visually, the hyper pigment patches are of a rippled pattern. When seen through a dermatoscopy, the pigmentation is seen to have a collection of dots, thin streaks and leaf-like bulging projections.
  • Studies have shown that 1 in 4 patients with macular amyloidosis can simultaneously have lichen amyloidosis.

Chapter 3: Macular Amyloidosis Diagnosis and Treatment

A macular amyloidosis diagnosis involves a thorough assessment of the patient’s medical history to identify any health disorders or hereditary factors that may have contributed to the condition.
If your dermatologist suspects you may be suffering from macular amyloidosis several lab tests may be conducted to confirm or rule out the diagnosis such as:

  • A skin biopsy - wherein a tissue sample is taken to test for amyloids and also rule out any other skin related disorders that are similar to macular amyloidosis.
  • Stain test - wherein a special compound is used on the tissue sample to check the characteristic appearance of amyloid deposits in the dermis.
  • Blood tests and Urine tests - these may be conducted to identify if the patient is suffering from an associated disease such as myeloma, a type of bone marrow tumour.

Unfortunately, primary localized cutaneous amyloidosis has no cure, no matter the type. Macular amyloidosis treatments are aimed at simply reducing its symptoms. Macular amyloidosis skin treatments emphasize reducing itching, and eliminating external triggers such as scratching, rubbing and friction in the affected skin areas of the patient.

Several treatments have been tested for macular amyloidosis but significant evidence of a treatment that is sure to cure the condition is yet lacking. Macular amyloidosis treatments, therefore, resemble those that are used to treat eczema. Some common treatments include:

  • Oral medications such as antihistamine tablets and certain immune-suppressing drugs.
  • Skin ointments such as corticosteroid creams and vitamin D ointments that help in flattening skin lesions, reducing irritation and hyperpigmentation.
  • Phototherapy treatment which involves a specific wavelength of ultraviolet light (UVB) commonly used to treat skin disorders
  • Dermabrasions, a skin resurfacing technique that is used to treat pigmentation.

Prevention and Self Care

Preventing macular amyloidosis is not always possible as sometimes it can be hereditary. However, reducing friction to the affected area and avoiding frequent rubbing or itching of the skin can prevent the aggravation of macular amyloidosis. It is recommended to do a full body checks from time to time to not only monitor your health but keep at bay any infections and risks that have the potential to cause macular amyloidosis. Our qualified dermatologists on MFine are able to recommend further preventive measures you can take to ensure your skin health is protected at all times.

Refer to more of our medical guides for helpful information on other health related conditions and their treatment.

Frequently asked questions:

  1. What causes macular amyloidosis?

Macular amyloidosis causes are currently unknown. This condition can be inherited from an older generation or due to environmental factors. Common triggers such as increased friction, scratching, itching and rubbing of the affected skin surface can aggravate macular amyloidosis in patients.

  1. What is the best treatment for pigmentation in macular amyloidosis?

Some skin pigmentation treatments that are used to treat hyperpigmentation caused by macular amyloidosis include specific oral medications, phototherapy, certain skin ointments and creams and dermabrasions. However, it is important to consult a dermatologist prior to carrying out any treatment. Dermatologists on MFine are available anytime, anywhere to provide you with an accurate diagnosis and the best treatment for pigmentation for your specific skin condition.

  1. What is the main cause of hyperpigmentation in the skin?

In macular amyloidosis, hyperpigmentation of skin is due to the build-up of abnormal proteins called amyloids on the skin. These occur in patches on the upper back and upper arm regions in a ripple-like design. These patches are usually dark brown, or greyish in color and are mildly itchy.

  1. What are some macular amyloidosis skin treatments?

Macular amyloidosis skin treatments closely resemble those that are used to treat eczema. They include oral medications in the form of antihistamines and steroids; certain skin ointments containing corticosteroids and vitamin D to reduce itching and hyperpigmentation; phototherapy and dermabrasions.

  1. Is dermabrasion a good skin pigmentation treatment in macular amyloidosis?

    Dermabrasions are a skin resurfacing technique used to originally treat scarring in the face but is now used as a common skin pigmentation treatment. In the case of macular amyloidosis, however, it is important to consult a dermatologist who will be able to prescribe the right treatment option for your symptoms.

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