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 in Indian patients. 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 hyperpigmentation differentiate primary localized cutaneous or skin amyloidosis into three forms:
1. Lichen amyloidosis
2. Nodular amyloidosis
3. Macular amyloidosis
What is Macular Amyloidosis in Indians?
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 skin hyperpigmentation in patches.
What makes Macular Amyloidosis different from Lichen Amyloidosis and Nodular Amyloidosis in Indians?
In the case of other primarily localized cutaneous amyloidosis types, for instance lichen amyloidosis, the skin hyperpigmentation 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 skin hyperpigmention 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 in India?
Several studies have shown that macular amyloidosis in India 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.
Learn more about macular amyloidosis from some of our top dermatologists on MFine today!