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Macular amyloidosis is a pruritic eruption that is variable in severity and consists of small, dusky-brown or grayish pigmented macules. Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly. CASE REPORT. An unusual presentation of macular amyloidosis. Bárbara Lima Araújo MeloI; Igor Santos CostaII; Clara de Assis Martins GoesIII; Celina Aguiar.

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No amyloid was detected around blood vessels or other cutaneous appendages. There are no clear guidelines for monitoring therapy for patients with macular amyloidosis. Gertz MA expert opinion.

The age of onset of MA for all patients ranged between 16 and 59 years. Family history is important because hereditary forms of macular amyloidosis do amilkidosis.

Click here for patient related inquiries. Check this box if you wish to receive a copy of your message. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. More studies are needed to compare different treatments and maintenance regimens. In cases of extensive or nodular cutaneous lesions it is important to carry out a systemic assessment.

The three main forms of primary localized cutaneous amyloidosis are lichen amyloidosis, macular amyloidosis, and nodular amyloidosis.


International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Enlarged tongue An enlarged tongue macroglossia can be a sign of amyloidosis.

Various systemic and cutaneous diseases in association with MA in our patients were chronic urticaria, diabetes mellitus, acne vulgaris, generalized maculxr, hypothyroidism, hypertension and idiopathic hirsutism. Macular amyloidosis simulating naevoid hyperpigmentation. Amyloidogenesis in organ-limited cutaneous amyloidosis: Frequent association between MEN 2A and cutaneous lichen amyloidosis.

J Eur Acad Dermatol Venereol. MA amiloidosie commonly associated with other skin diseases, such as atopic dermatitis. In general, amyloidosis is caused by the buildup of an abnormal protein called amyloid.

Unusual pigmentation on the skin over trunk bones and extremities. Histopathological examination of the cutaneous lesion revealed significant quantities of an amyloid deposit in the papillary dermis, which could be more clearly observed after Congo red staining, as well as foci of pigmentary incontinence. It is the zmiloidosis subtle form of cutaneous amyloidosis and tends to persist unchanged for many years. In some families with the hereditary forms of primary localized cutaneous amyloidoses, an association with multiple endocrine neoplasia MEN type 2A, also known as Sipple syndrome, has been established.


Amyloid is an abnormal protein that is produced in your bone marrow and can be deposited in any tissue or organ. Mayo Foundation for Medical Education and Research; J Am Acad Dermatol. For want of the requisite information on the natural course and definitive etiology the disease, MA remains an enigma and a source of concern for the suffering zmiloidosis and physicians.


Discussion Amyloidosis is an extra-cellular deposition of the fibrous protein either involving multiple organ systems systemic amyloidosis or restricted to a single-tissue site localized amyloidosis.

A Clinico-Epidemiological Study of Macular Amyloidosis from North India

MA classically presents in a rippled pattern or as reticulate pigmentation. Indian J Dermatol Venerol Leprol. In order to differentiate macular amyloidosis aniloidosis other skin disorders, the presence of amyloid must be confirmed. Request an Appointment at Mayo Clinic.

Courtesy of Bryan Anderson, MD Expected results of diagnostic studies A skin biopsy stained with hematoxylin and eosin typically demonstrates amorphous deposits of eosinophilic material amyloid deposits within the papillary dermis.

Touart, D, Sau, P. In our study, there was a wide variation in the duration of the disease ranging from 1 month to 12 years.

A Clinico-Epidemiological Study of Macular Amyloidosis from North India

The literature on the subject is also reviewed. Who is at Risk for Developing this Disease? A hyperpigmented, rippled eruption in a Hispanic woman: Histochemistry and electron microscopy. Dermabrasion, CO2 laser, and pulse dye laser provide alternative options for patients unresponsive to other treatments.

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