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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Laboratory testing is not recommended for macular amyloidosis because there are amiloidodis specific laboratory abnormalities associated with the condition. Typical histological findings are an amorphous amyloid deposit in the papillary dermis between the interpapillary pegs, where foci of pigmentary incontinence can frequently be found.
The literature on the subject is also reviewed.
Macular Amyloidosis (Friction Amhyloidosis)
Palitz LL, Peck S. Other search option s Alphabetical list. Health care resources for this disease Expert centres 22 Diagnostic tests 1 Patient organisations 2 Amlloidosis drug s 0. Only comments written in English can be processed.
This study attempts to evaluate the epidemiology and risk factors in the etiology of MA. There are no clear guidelines for monitoring therapy for patients with macular amyloidosis. Patients can sometimes have physical findings of both lichen amyloidosis and macular amyloidosis, known as biphasic amyloidosis. Identification of gammaglobulins and C3 in the lesions by immunofluorescence. 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.
Macular amyloidosis simulating naevoid hyperpigmentation. Amiloldosis timing of follow-up care is based on the individual’s response to a chosen treatment. Lamina densa malformation involved in histogenesis of primary localized cutaneous amyloidosis. Though the exact incidence of MA is not known, it is by no means an uncommon disease. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.
Lack of effect of dimethylsulphoxide in cutaneous amyloidosis. Another report involving a patient who was treated with NB UVB three times per week showed marked improvement after 5 months of treatment.
Clinically, the lesions in all of our patients presented as hyperpigmented macules, predominantly in a rippled pattern. Disease definition Macular amyloidosis MA is a rare chronic form of cutaneous amyloidosis see this term mafular, a skin disease 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.
Complete history and physical examination to exclude any associated systemic disorder or drug usage leading to cutaneous pigmentation was obtained. Histopathology of the tissue specimens corroborates previous studies that the use of special stains increases the sensitivity of detecting amyloid in more specimens. Macular amyloidosis typically presents as gray-brown pruritic macules, which gradually mscular into patches with amiolidosis rippled pattern involving the upper back Figure 1 and less often the arms, chest and thighs.
Congo Red shows apple-green birefringence under polarized light Methyl or cresyl violet Thioflavin T with examination using fluorescence microscopy.
Orphanet: Amiloidosis macular
Unusual Clinical Scenarios to Consider in Patient Management There is an unclear link between primary localized cutaneous amyloidoses and a variety of immune disorders including systemic sclerosis, CREST syndrome, rheumatoid arhritis, systemic lupus erythematosus, primary biliary cirrhosis, autoimmune cholangitis, Kimura disease, ankylosing aamiloidosis, autoimmune thyroiditis, IgA nephropathy, and sarcoidosis.
Family history is important because hereditary forms of macular amyloidosis do exist.
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.
Brownstein MH, Hashimoto K. The amyloid deposits in macular amyloidosis are keratinocyte-derived. Sun exposure did seem to play an important role in the localization of disease. Diffuse primary cutaneous amyloidosis. This is a case of macular amyloidosis with an unusual presentation. Laboratory findings were as follows: The use of nylon towels or brushes on the skin has also been described as a risk factor for the development of MA.
Nylon cloth macular amyloidosis. Results The prevalence of MA in our out-patient clinic was observed to be 0. Intermittent use of topical dimethyl sulfoxide in macular and papular amyloidosis. Contrary to previous reports, MA in our study was observed in a majority of patients of comparatively lighter skin than in those with a higher skin phototype.
In the patient studied here this failed to reveal any abnormal results.
Journal List Indian J Dermatol v. Encouraging results were reported with the use of Q-switched Nd: Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated.
Accessed May 16, aimloidosis Diffuse biphasic cutaneous amyloidosis. Overview Amyloidosis am-uh-loi-DO-sis is a rare disease that occurs when a substance called amyloid builds up in your organs.
Shanon J, Sagher F. Occasionally, more amiolidosis of necrotic keratinocytes were found overlying the subepidermal amyloid deposits.
A Clinico-Epidemiological Study of Macular Amyloidosis from North India
Etiology Pathophysiology The amyloid deposits in macular amyloidosis are keratinocyte-derived. There was also mild pigmentary incontinence, resulting in the hyperpigmentation observed clinically.
Amyloidosis of the skin.