Macular Amyloidosis(Cutaneous amyloidosis)
What is Macular Amyloidosis? What are its signs and symptoms? What are its complications? What is its differential diagnosis? What is the treatment?
What is Macular Amyloidosis?
Macular amyloidosis is a pruritic eruption that is variable in severity and consists of small, dusky-brown, or greyish pigmented macules distributed symmetrically over the upper back and, in some patients, the arms.
Who is more prone to get affected?
1. It mainly affects the adult population.
2. Women are more prone compared to men.
3. It is most common in Asians, Middle Eastern, and South Americans.
What are the signs and symptoms?
It is a pruritis condition, usually small dusky brownish pigmented eruptions are distributed on the upper back and occasionally spreads to the arm.
General symptoms of amyloidosis may include:
- Changes in skin color
- Severe fatigue
- Feeling of fullness
- Joint pain
- Low red blood cell count (anemia)
- Shortness of breath
- Swelling of the tongue
- Tingling and numbness in legs and feet
- Weak hand grip
- Severe weakness
What happens inside the body when a person has macular amyloidosis?
The amyloid deposits in macular amyloidosis are keratinocyte-derived. The exact pathogenesis leading to the apoptosis of keratinocytes is not clear but trauma such as friction (rubbing and scratching), genetic predisposition, infectious agents, and ultraviolet radiation are possible causes.
What are the various treatments available and which one will help? There are various treatments such as –
- Topical corticosteroids
- Topical calcineurin inhibitors
- Intralesional corticosteroids
- Systemic retinoids
- Systemic cyclophosphamide
Physical modalities
- Ultraviolet (UV) B phototherapy (narrowband and broadband)
- PUVA (psoralen with UVA) phototherapy
Surgical options
- Dermabrasion
- CO2 laser therapy
Homeopathy
It is the best option that is safe and can be taken for a long duration without affecting your vital organs as the body develops resistance when corticosteroids are taken for a longer duration and there are several side-effects that hamper vital organs of the body.
What are the complications?
Macular amyloidosis has not been reported to progress to systemic disease with the amyloid deposits exclusively localized to the skin. However, there is evidence linking primary localized cutaneous amyloidosis to a variety of immune disorders including systemic sclerosis, CREST syndrome, rheumatoid arthritis, systemic lupus erythematosus, primary biliary cirrhosis, autoimmune cholangitis, Kimura disease, ankylosing spondylitis, autoimmune thyroiditis, IgA nephropathy, and sarcoidosis.
In some families with the hereditary forms of primary localized cutaneous amyloidosis, an association with multiple endocrine neoplasias (MEN) type 2A, also known as Sipple syndrome, has been established. MEN type 2A is an autosomal dominant disorder including the triad of medullary carcinoma of the thyroid, pheochromocytoma, and hyperparathyroidism.
What if a person does not take any medicines?
The itching becomes unbearable and it deteriorates the quality of day-to-day life, constant itching leads to further friction which aggravates the condition, so it is a vicious cycle that gets in control with the help of medication.
What investigation is needed to confirm the diagnosis?
Skin biopsy can be conclusive.
Congo red stains can demonstrate skin amyloid deposits.
What is the differential diagnosis?
-Pediatric atopic dermatitis
-Poikiloderma of civatte
-Postinflammatory hyperpigmentation
Can a person live a normal life with such a condition?
Yes, Once the itching comes in control the quality of life improves. Hyperpigmentation can be helped by cosmetic concealers to help patients gain self-confidence.
What are the dietary restrictions in this condition?
-Omega-3 fatty acids (Prescribed dosage is recommended to avoid side effects)
-Vitamin C
How does this condition spread?
It is not contagious, it does not spread by touching the patient. It may pass on genetically from parent to child.
Which are commonly indicated Homoeopathic medications?
In such conditions, constitutional medicine works the best. However, few commonly indicated medicines are: –
-Hepar Sulp
-Sulphur
-Graphites
-Mezereum
