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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Primary cutaneous amyloidosis – Wikipedia
Various systemic and cutaneous diseases in association with MA in our patients were chronic urticaria, diabetes mellitus, acne vulgaris, generalized xeroderma, hypothyroidism, hypertension and idiopathic hirsutism. Histopathology of the tissue specimens corroborates previous studies that the use of special stains increases the sensitivity of detecting amoloidosis in more specimens.
MA classically presents in a rippled pattern or as reticulate pigmentation. This content does not have an Arabic version.
The use of nylon towels or brushes on the skin has also been described as a risk factor for the development of MA. Dermatological examination revealed irregular, reddish-brown, lace-like macules on the outer face of the arms, back of the neck, sacral region and legs. There is a strong association between localized cutaneous amyloidosis and notalgia paresthetica.
In some families with the hereditary forms of primary amiloiddosis cutaneous amyloidoses, an association with multiple endocrine neoplasia MEN type 2A, also known as Sipple syndrome, has been established.
Pathology Outlines – Amyloidosis
MEN type 2A is an autosomal dominant disorder including the triad of medullary carcinoma of the thyroid, pheochromocytoma, and hyperparathyroidism. Though the exact incidence of MA is not known, it is by no means an uncommon disease. In one study, acitretin 0. Amiloidosie, sunlight could be incriminated as a risk factor in the causation of MA in our patients.
You may not experience signs and symptoms of amyloidosis until the condition is advanced.
Ferri’s Clinical Advisor Epidemiologymacular amyloidosisrisk factors. Patients and family members should be informed about the chronicity of the disease and the potential for trying multiple therapies before resolution of symptoms. Role of friction was inconclusive.
Indian J Dermatol Venerol Leprol.
Apart from the considerable cosmetic disability, patients occasionally complain of troublesome pruritus. Most cases can be treated with strong topical corticosteroids, normally for a short period. Poikiloderma-like cutaneous amyloidosis in an ethnic Chinese girl.
A Clinico-Epidemiological Study of Macular Amyloidosis from North India
Amyloid is an abnormal protein that is produced in your bone marrow and can be deposited in any tissue or organ. Disease definition Macular amyloidosis MA is a rare chronic form of cutaneous amyloidosis see this terma skin a,iloidosis characterized by the accumulation of amyloid deposits in the dermis, clinically characterized by pruritic hyperkeratotic gray-brown macules that give a rippled or reticulated pattern of pigmentation usually in the upper back and extensor sites of arms, forearms and legs, and histologically by the deposition of amyloid in the upper dermis and close to the basal cell layer of the epidermis.
Upper back was involved in 40 patients. Similar observations were made in studies by Rasi et al. A Venereol distinct subset of macular amyloidosis. How to cite this article. Therapeutic removal of amyloid deposits in cutaneous amyloidosis by localised intra-lesional injections of anti-amyloid antibodies.
The patient did not present with any systemic complaints, and there were no abnormal findings on physical examination other than those already described. Dermabrasion, CO2 laser, and pulse dye laser provide alternative options for patients unresponsive to other treatments.
Comparative study of phototherapy UVB macula photochemotherapy PUVA vs topical steroids in the treatment of primary cutaneous lichen amyloidosis. It is essential that any individual in such a kindred who presents with either macular amyloidosis or lichen amyloidosis be assessed for the RET protooncogene; if the mutation is present, a prophylactic thyroidectomy should be strongly considered.
These changes did not always correlate with the deposition of amyloid and extended well beyond the areas of amyloid deposits.
However, small amounts of amyloid may not be detected by methyl violet and Congo red staining, which can complicate the diagnosis of macular amyloidosis.
Macular form of cutaneous amyloidosis was first described by Palitz and Peck in A study of amiloieosis cases with special reference to the role of the epidermis in its histogenesis. Sign up for our Email Newsletters. In general, amyloidosis is caused by the buildup of an abnormal protein called amyloid.
Furthermore, itching, which is common amiloidpsis PCAs, may not be present in the macular form, as in this case. Palitz LL, Peck S. J Am Acad Dermatol. Click here for information on linking to our website or using our content or images. Some people with amyloidosis experience purpura — a condition in which small blood vessels leak blood into the skin, causing purplish patches. In amiloidozis study comprising 50 patients from Northern India, we studied the possible etiological and risk factors of MA.
Additionally, there was focal disruption of the basal layer with pigmentary incontinence in only a few cases. A hyperpigmented, rippled eruption in a Hispanic woman: No amyloid was detected around blood vessels or other cutaneous appendages. 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.
Macular Amyloidosis (Friction Amhyloidosis)
Diffuse primary cutaneous amyloidosis. Open in a separate window. Accessed December 31st, There are also reports of rare hypochromic and poikilodermatous forms 4.
When stained with Congo red and viewed under polarized light, the amyloid substance exhibits clear birefringence. This produced a significant reduction in pigmentation, which is considered by MA patients to be the most disturbing aspect of the condition.