Presentation
In one of the two cases lichen amyloidosis of the auricular concha reported here, concomitant macular amyloidosis of the back was present. [dermatology-s10.cdlib.org]
Herrera, Ahmet Dogan Humana Press, ١٧/٠٨/٢٠١٥ - 542 من الصفحات The second edition of this text presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [books.google.com]
LCD type II (Meretoja syndrome) is a systemic disease presenting in young adulthood with cutaneous, nervous and corneal manifestations. Frequent recurrent erosions and impaired corneal sensation are often present by age 40 years. [healio.com]
Macroglossia with tooth indentation was present in second case. Hepatomegaly occurs in 50% of patients and splenomegaly in 10%. Hepatomegaly was present in our case 1. [odermatol.com]
Heat intolerance and telangiectases of the ears are often present early in the course of the disease. 11 The characteristic angiokeratomata develop after puberty and present as tiny red–black bilaterally symmetrical papules, 0.5–2 mm in diameter, with [basicmedicalkey.com]
Respiratoric
- Hoarseness
The vocal cords may also be affected, causing hoarseness of voice. Endocrine organs such as the thyroid gland and adrenal glands are rarely involved. [news-medical.net]
Systemic amyloidosis The signs and symptoms of primary systemic amyloidosis are generally non-specific and include: Fatigue Weight-loss Oedema Shortness of breath Lightheadedness Numbness, tingling sensation Hoarseness. [dermnetnz.org]
Primary, and myeloma-associated, systemic amyloidosis The mean age of onset is 65 years, with a slight male predominance The signs and symptoms are generally non-specific and include itch, fatigue, weight-loss, shortness of breath, a hoarse voice, numbness [pcds.org.uk]
Some of the more common findings during a review of systems include the following: weight loss, weakness/fatigue, dyspnea, edema, paresthesias, light-headedness, syncope, constipation/diarrhea, and hoarseness/change of voice. [dermatologyadvisor.com]
- Cough
Periorbital purpura (raccoon eyes) can be elicited by increased intrathoracic pressure as a result of the Valsalva maneuver and coughing. Amyloid infiltration of blood vessels also manifests as petechiae and ecchymoses. [dermatologyadvisor.com]
Periorbital pupura may form following coughing or the valsalva maneuver. When amyloid infiltrates travel to the dermis, subcutaneous nodules or plaques may form. The lesions appear hemorrhagic, though may also appear smooth and waxy. [bobbybukamd.com]
Liver, Gall & Pancreas
- Jaundice
壊血病 scorbutus 核黄疸 Kernikterus (独), kernicterus, nuclear jaundice 家族性アミロイドーシス familial amyloidosis 家族性アミロイドポリニューロパチー familial amyloidotic polyneuropathy 家族性LCAT欠損症 familial lecithin cholesterol acyltransferase deficiency 家族性高コレステロール血症 familial hypercholesterolemia [jsge.or.jp]
She also had a 1cm x 1cm healing ulcer on the left medial calf and showed no evidence of jaundice, lymphadenopathy, or hepatosplenomegaly. Her ankle brachial pressure index was 0.8 bilaterally. [woundsresearch.com]
Musculoskeletal
- Osteoporosis
[…] collagen disease 甲状腺機能亢進症 hyperthyroidism 甲状腺機能低下症 hypothyroidism 甲状腺中毒症 thyrotoxicosis 後天性免疫不全症候群 acquired immune deficiency syndrome 高コレステロール血症 hypercholesterolemia 高トリグリセリド血症 hypertriglyceridemia 高リポ蛋白血症 hyperlipoproteinemia ゴーシェ病 Gaucher disease 骨粗鬆症 osteoporosis [jsge.or.jp]
Skin
- Pruritus
[…] in pruritus, pigmentation or flattening of papules was labeled as 'no response'. [idoj.in]
Clinically-Relevant Mutations and Pathophysiology Disease: Primary cutaneous amyloidosis Description: A skin condition characterized by pruritus, skin scratching and by deposits of amyloid in the dermis. [guidetopharmacology.org]
The patient reported a consistent reduction of pruritus at the end of treatment, from 9 to 2 in a 0 to 10 visual analogue scale (VAS). [clinicaldermatology.eu]
Typically, in both pedigrees, the onset of symptoms was around puberty, and pruritus usually began on the lower legs. [ncbi.nlm.nih.gov]
Affiliated tissues include skin, and related phenotypes are scaling skin and pruritus Genetics Home Reference : 26 Primary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin, [malacards.org]
- Hyperpigmentation
Macular amyloidosis is characterized by small pigmented macules that may merge to produce macular hyperpigmentation, sometimes with a reticulate or rippled pattern. [malacards.org]
She also noticed reticular hyperpigmentation macules surrounding the spotty hypopigmentation. [bmcdermatol.biomedcentral.com]
Cutaneous exam also revealed macular hyperpigmentation in a rippled pattern over her forearms, shoulders, back, buttocks, and upper thighs (Figure 1). [woundsresearch.com]
- Dermatitis
Stay up to date with recent society guidelines, including the latest from the American Academy of Dermatology, covering a variety of conditions such as melanoma and atopic dermatitis. [books.google.com]
Acta Dermato-Venereologica covers inter alia: ~ Atopic dermatitis and contact allergy ~ Facial dermatoses and adnexal disorders ~ New treatments ~ Psoriasis and genodermatoses ~ Psychodermatology ~ Sexually transmitted diseases ~ Skin biology and inflammation [ingentaconnect.com]
It is sometimes associated with other conditions such as atopic dermatitis, sarcoidosis, and psoriasis. [rarediseases.info.nih.gov]
The type of dermatitis is generally determined by the person's history and the location of the rash. [clinicaldermatology.euroscicon.com]
This treatment have been used in three patients with good results, and in two of those cases lichen amyloidosis was associated with atopic dermatitis ( 3, 5, 17 ). [clinicaldermatology.eu]
- Purpura
Cutaneous features include petechiae, purpura and alopecia. [pcds.org.uk]
Amyloid purpura. N Engl J Med 2007; 356:2406. Masuda C, Mohri S. Nakajima. Histopathological and immunohistochemical study of amyloidosis cutis nodularis atrophicans. Comparison with systemic amyloidosis. Br J Dermatol ;119(1): 33-43. [revistas.unicartagena.edu.co]
Amyloid infiltration of vessel wall causes capillary wall fragility, which leads to purpura and ecchymosis after a minor trauma or even spontaneously. Periorbital area is one of the common sites of expression of purpura. [odermatol.com]
One of the most classic skin lesions is purpura, which can occur after minor trauma (pinch purpura). Periorbital purpura (raccoon eyes) can be elicited by increased intrathoracic pressure as a result of the Valsalva maneuver and coughing. [dermatologyadvisor.com]
Skin and soft tissues - Primary amyloidosis may affect the skin and soft tissues and cause blood spots called purpura, itching and rashes. Lungs and vocal cords – In primary amyloidosis, deposits often accumulate in the lungs. [news-medical.net]
- Urticaria
[…] contact allergy ~ Facial dermatoses and adnexal disorders ~ New treatments ~ Psoriasis and genodermatoses ~ Psychodermatology ~ Sexually transmitted diseases ~ Skin biology and inflammation ~ Skin cancer and pigmentation ~ Skin immunology and lymphoma ~ Urticaria [ingentaconnect.com]
[…] effusions& pain Myelopathy β 2 -microglobulin Amyloidosis, renal (ALys) Renal Visceral Lysozyme (LYZ) AApoAIIRenalApolipoprotein A II (APOA2) AApoCIIRenalApolipoprotein C II (APOC2) AApoCIIIRenalApolipoprotein C III (APOC3) Muckle-Wells syndrome Cold urticaria [neuromuscular.wustl.edu]
Cerebrovascular amyloidosis Conjunctival amyloidosis Cutaneous amyloidosis Danish type familial amyloid cardiomyopathy Dilated cardiomyopathy secondary to amyloidosis Dominant primary localized cutaneous amyloidosis Familial amyloid nephropathy with urticaria [icd9data.com]
Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. J Am Acad Dermatol. 2003; 49 :679–84. 19. Gambichler T, Terras S, Kreuter A. [clinicaldermatology.eu]
However, contact urticaria has not been reported in our study. Cutaneous amyloidosis is a disease of middle-aged persons with a female preponderance. [idoj.in]
Urogenital
- Incontinence
The dermal changes were lymphohistiocytic infiltrates (50%), pigment incontinence (22.72%), and increased dermal collagen (18.2%). [pigmentinternational.com]
The histological features which were present in all patients were mild hyperkeratosis, atrophy of the stratum malpighii, increased basal layer pigmentation, dermal pigmentary incontinence and dense condensed collagen in the papillary dermis. [cags.org.ae]
Focal disruption of the basal cell layer with pigment incontinence and melanophages in the papillary dermis were seen in four (15%) biopsy specimens. [e-ijd.org]
[…] macrophages or fibroblasts, 2 as some have noted dermal fibroblasts in proximity to amyloid deposits containing amyloid-like filaments. 2 It has also been hypothesized that perhaps basal cells at the epidermo-dermal junction produce the amyloid. 11 Pigmentary incontinence [woundsresearch.com]
Workup
Colonoscopy
- Colitis
It is estimated that about 5% of patients with ulcerative colitis develop pyoderma gangrenosum. In some patients, pyoderma gangrenosum may develop years before the onset of inflammatory bowel disease. [bobbybukamd.com]
Treatment
A comprehensive review of the literature on the treatment options for PCA showed no controlled trials available. However, case series indicated phototherapy (UVB, narrow band UVB, photochemotherapy) as the most successful treatment. [clinicaldermatology.eu]
What is the treatment of systemic amyloidosis? Nonspecific treatment Treatment is directed at maintaining the function of affected organs. For example, kidney failure can be treated with dialysis and congestive heart failure with diuretics. [dermnetnz.org]
Broaden your knowledge with updated information on serological diagnosis of pemphigus, TNF-I for hidradenitis suppurativa, the use of immunosuppressives for atopic dermatitis, excimer laser for the treatment of vitiligo and much more. [books.google.com]
Allocation: Non-Randomized Intervention Model: Parallel Assignment Masking: Single (Outcomes Assessor) Primary Purpose: Treatment Official Title: Efficacy of Methylene Blue Mediated Photodynamic Therapy for Primary Localized Cutaneous Amyloidosis Treatment [clinicaltrials.gov]
Prognosis
Nodular amyloidosis is extremely rare, and there are notable differences in clinical presentation, prognosis, histology, and pathogenesis between this entity and the macular and lichenoid variants. [actasdermo.org]
Dialysis-related amyloidosis Pathophysiology Systemic Amyloidosis Organ-specific Amyloidosis Microscopic Pathology Causes Differentiating Amyloidosis from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis [wikidoc.org]
Topics covered include classification/epidemiology, pathogenesis, clinical features (with classic images), diagnosis, prognosis and treatment. [dermatologyadvisor.com]
Prognosis depends upon the extent of involvement. Treatment of amyloidosis is aimed at reducing the supply of precursor proteins [ 1 ]. [odermatol.com]
Etiology
Herrera, Ahmet Dogan Humana Press, ١٧/٠٨/٢٠١٥ - 542 من الصفحات The second edition of this text presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [books.google.com]
Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. [e-ijd.org]
However, the etiology of amyloidosis remains unclear and population-based studies would prove to be of great benefit in evaluating the exact etiology of amyloidosis. [idoj.in]
It is proposed that ApoE might play an important part in the etiology of amyloidosis by functioning as a chaperone to promote the formation of amyloid filaments. [cags.org.ae]
Its etiology is unknown but chronic irritation to the skin has been proposed as an etiological factor. [4] The amyloid deposits in LA are mainly restricted to the upper dermis and arise because of focal epidermal damage with subsequent conversion of necrotic [ijdvl.com]
Epidemiology
Clinico-epidemiological study of macular amyloidosis from North India. Indian J Dermatol, 57(4), 269-74. Beccia, M., Biver, T., & Pardini, A. (2012). [recimundo.com]
The present study was an attempt to study the epidemiology of PLCA and the effectiveness of DMSO on the lesions. [idoj.in]
“The epidemiology of AL and AA amyloidosis”. Baillieres Clin Rheumatol. vol. 8. 1994. pp. 627-34. [dermatologyadvisor.com]
Epidemiology of primary cutaneous amyloidoses in southeast Asia. Clin Dermatol 1990;8:20-4. 13. Ollague W, Ollague J, Ferretti H. Epidemiology of primary cutaneous amyloidoses in South America. Clin Dermatol 1990;8:25-9. 14. [pigmentinternational.com]
Keywords: Epidemiology, macular amyloidosis, risk factors How to cite this article: Bandhlish A, Aggarwal A, Koranne RV. A clinico-epidemiological study of macular amyloidosis from North India. [e-ijd.org]
Pathophysiology
The genetic basis of FPLCA involves mutations in the OSMR and IL31RA genes but the disease pathophysiology is not fully understood. [ncbi.nlm.nih.gov]
Clinically-Relevant Mutations and Pathophysiology Disease: Primary cutaneous amyloidosis Description: A skin condition characterized by pruritus, skin scratching and by deposits of amyloid in the dermis. [guidetopharmacology.org]
Amyloidosis Main page Overview Historical Perspective Classification AL amyloidosis AA amyloidosis Heritable amyloidosis Age-related (senile) systemic amyloidosis Dialysis-related amyloidosis Pathophysiology Systemic Amyloidosis Organ-specific Amyloidosis [wikidoc.org]
Subtrack Ultraviolet Radiatioon Exposure Genetics and Mutations Diagnosis and Biopsy Pathophysiology Hyperpigmentation | Der matology Conferences | Cosmetology Conferences | Plastic Surgery Conferences | Dermatology Meetings | Dermatologist Conferences [clinicaldermatology.euroscicon.com]
Etiology Pathophysiology The amyloid deposits in macular amyloidosis are keratinocyte-derived. [infectiousdiseaseadvisor.com]
Prevention
Within this we support a broad spectrum of research with the objective of preventing disease and disability and of alleviating physical disability. [action.org.uk]
Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies v t e Urinary system · Pathology · Urologic disease / Uropathy ( N00-N39, 580-599 ) Abdominal Nephropathy / ( nephritis + nephrosis ) Glomerulopathy / glomerulitis [wikidoc.org]
Sub track Prevention and Management Genital wart Flat wart Periungual wart Actinic keratosis | Dermatology Conferences | Cosmetology Conferences | Plastic Surgery Conferences | Dermatology Meetings | Dermatologist Conferences 2019 Actinic keratosis is [clinicaldermatology.euroscicon.com]
The goal of treatment is to prevent secondary infection and to allow the lesions to heal on their own. [bobbybukamd.com]