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Macular amyloidosis

What is it macular amyloidosis?

Macular amyloidosis is one of three forms of primary located cutaneous amyloidosis. a proteinaceous material amyloid – is deposited harmlessly on the skin, causing hyperpigmented patches

Macular amyloidosis

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Who gets macular amyloidosis?

Most cases of macular amyloidosis arise in early adulthood. there is a major incidence of macular amyloidosis in people of Asian, Middle Eastern, and South American descent than in people of European and North American descent [1]. Populations with darker skin (Fitzpatrick skin phototype IV and V) are more commonly affected.

Up to 10% of macular amyloidosis cases are family in origin.

  • An inherited form of localized primary cutaneous amyloidosis has been identified in Brazilian and Chinese families due to a mutation at OSMR gene [2,3].
  • A autosomal Dominant primary localized cutaneous amyloidosis is related to a mutation in the IL-31RA protein-coding gene, interleukin-31 receiver A [2].

Women present macular amyloidosis more frequently than men; This may be due to women seeking medical attention earlier than men due to cosmetic problems.

associations with multiple endocrine neoplasm type 2A (MEN2A) and atopic dermatitis has been reported [4,5].

What causes macular amyloidosis?

The precise cause of macular amyloidosis is uncertain, although there appears to be an interaction between genetic and environmental factors that are triggered by prolonged periods of friction, rubbing, or scratching of the affected region.

Two theories have been proposed to explain the aberrant statement of amyloid: the fibrillary body theory [6,7] and the secretory theory [8].

What are the clinical features of macular amyloidosis?

Macular amyloidosis presents with small, dark brown or bluish-gray hyperpigmented macules.

  • The macules are symmetrically distributed over the upper back (especially the interscapular area) and the extensor muscle upper arm surfaces.
  • Uncommon, the face, neck, collarbones, breasts, and armpits They are involved.
  • Macular amyloidosis is usually pruritus, although the degree of pruritus varies from mild to severe.
  • It can take on a subtle wavy or lattice pattern, which resembles the “seafloor pattern” seen in lichen amyloidosis.
  • Macular amyloidosis may coexist with lichenyloid in a 25% of affected patients. [9].

Dermoscopy reveals a central white or brown area surrounded by brown pigmentation as fine radiating streaks, dots, leaf-like projections, and bulbous projections [10].

How is macular amyloidosis diagnosed?

The diagnosis of macular amyloidosis is based on the characteristic clinical presentation and histopathological findings of cutaneous amyloid deposits within a skin biopsy. See primary cutaneous amyloidosis pathology.

  • Amorphous eosinophilic material is deposited extracellularly in the papillary dermis and is accompanied by dermal melanophages.
  • Congo red staining may reveal positive apple-green birefringence under polarized light, which is characteristic of amyloid. [9]. This birefringence is not observed in all cases. [9].
  • The distinction between lichen amyloidosis and macular amyloidosis is related to the amount, appearance, and location of the deposition.
  • Histopathology could also demonstrate slight hyperkeratosis, hypergranulosisand subepidermal cleft. Cicada bodies can be seen in the basal cap of the epidermis.

Which is the differential diagnosis for macular amyloidosis?

Macular amyloidosis can be confused with other pigment disorders, including:

  • Atrophic lichen planus
  • Drug induced hyperpigmentation
  • Erythema dyschromicum perstans
  • Notalgia paraesthetica
  • Photocontact dermatitis
  • Poikiloderma by Civatte
  • Post-inflammatory hyperpigmentation
  • Pityriasis versicolor

What is the treatment for macular amyloidosis?

The main goal of treatment is relief of itching.

The importance of avoiding mechanical stressors such as friction, rubbing or scratching of the affected regions should be emphasized.

A standardized treatment regimen has not been established. [eleven]. In most cases, current corticosteroids and emollients are used to reduce itching.

Other local medications may include:

  • Topical calcineurin inhibitors (tacrolimus and pimecrolimus)

  • intralesional corticosteroids.

Systemic medications are rarely required; however, macular amyloidosis has been reported to be successfully treated with the following medications:

  • Cyclophosphamide
  • Cyclosporine
  • Dimethyl sulfoxide (DMSO) [12–14]

  • Systemic retinoids [15].

Physical therapies reported to be beneficial include:

  • Dermabrasion
  • Occlusive dressings
  • Transcutaneous electrical nerve stimulation (TENS) [16]
  • PUVA (photochemotherapy)

  • UVB phototherapy [17]

  • Fractional To be treatment [18]

  • Nd:YAG laser (532nm and 1064nm) [19,20]

  • Pulsed dye laser treatment [21].

What is the result of macular amyloidosis?

Macular amyloidosis is persistent and can have a significant impact on quality of life due to cosmetic disfigurement and persistent itching. [22].