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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Pathology Outlines – Amyloidosis

Congo Red staining left and Thioflavin-T staining with fluorescent microscopy right. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis. Additional information Further information on this disease Classification s 1 Gene s 0 Other website s 0.

However, there is evidence linking primary localized cutaneous amyloidoses 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 smiloidosis. Severe amyloidosis can lead to life-threatening organ failure. Ferri’s Clinical Advisor Etretinate for the treatment of lichen amyloidosis.

Nylon cloth macular amyloidosis.

Histopathological examination of the cutaneous lesion revealed significant quantities of an amyloid deposit in the papillary dermis, which could be more clearly amiloidowis after Congo red staining, as well as foci of pigmentary incontinence.

Touart DM, Sau P. Lack of effect of dimethylsulphoxide in cutaneous amy loidosis. Page views in Two theories, fibrillar body theory and secretory theory, have been proposed.

Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Rajagopalan K, Tay CH. Genetic factors in the amyloid diseases.


Primary cutaneous amyloidosis

Gertz MA expert opinion. There is a strong association between localized cutaneous amyloidosis and notalgia paresthetica. In general, the treatment of PLCA is disappointing. Histopathology of primary cutaneous amyloidoses and systemic amyloidosis.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Upper back was involved in 40 patients.

For want of the requisite information on macukar natural course and definitive maculae, the disease MA remains an enigma and a source of concern for the suffering patients. Histopathological findings of an amyloid deposit in the dermal papillae close to the basal layer of the epidermis confirmed the diagnosis Figure 5.

In a study by Ostovari et al.

YAG laser therapy for reduction of pigmentation in macular amyloidosis patches. Maurer MS, et al. The literature on the subject is also reviewed. Lesions are characterized by lace-like, reddishbrown stains usually located on the upper back and, more infrequently, on the arms, chest and legs. Hashimoto K, Kobayashi H. Fifty patients with a clinical diagnosis of MA attending the out-patient clinic of a tertiary care dermatology center were enrolled in the study.

American Journal of Hematology. But treatments can help you manage your symptoms and limit the production of amyloid protein. However, as treatment follow-up lasted only two months, possible side effects such as residual hypopigmentation were not identified, particularly in patients with phototypes V or VI, for whom six months follow-up after the multiple laser sessions would be required.

A study by Clos et al. You must be a registered member of Dermatology Advisor to post a comment. A case report of the tumefactive variant and a review of the spectrum of clinical presentations”.


Additionally, there was focal disruption of the basal layer with pigmentary incontinence in only a few cases. A clinicopathologic study from Saudi Arabia. Amyloidosis am-uh-loi-DO-sis is a rare disease that occurs when a substance called amyloid builds up in your organs. Clinical resolution with cyclosporine.

An antigenic identity between epidermal keratin and skin amyloid. When stained with Congo red and viewed under polarized light, the amyloid substance exhibits clear birefringence. Accessed April 27, See your doctor if you persistently experience any of the signs or symptoms associated a,iloidosis amyloidosis.

Macular Amyloidosis (Friction Amhyloidosis)

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.

Primary localized cutaneous amyloidosis. There were no systemic findings. Laboratory findings were as follows: There was mild hyperkeratosis of the epidermis in three Identification of gammaglobulins and C3 in the lesions by immunofluorescence. Occasionally, more number of necrotic keratinocytes were found overlying the subepidermal amyloid deposits. The observation about the role of friction could not be conclusively supported or negated.

Histology revealed amyloid deposits in the papillary dermis which exhibited apple-green birefringence after Congo red staining. A recently discovered cytokine, interleukin ILhas been proposed macylar play a role in itching[ 49 ] and has been implicated in the pathophysiology of itchy dermatosis such as PLCA sporadic and familial.

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