Symptoma

Familial Primary Localized Cutaneous Amyloidosis

FPLCA

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]

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]

Although most cases occur sporadically in people with no family history of the condition, there are rare familial forms caused by changes ( mutations ) in the OSMR or IL31RA gene. [3] [4] Treatment is based on the signs and symptoms present in each person [rarediseases.info.nih.gov]

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]

  • Pathologist

    Amyloid and Related Disorders, Second Edition will be invaluable to specialized and general pathologists as well as cytopathologists. Other medical professionals may also benefit from this concise update on the systemic amyloidoses. [books.google.com]

    The term amyloid was reportedly coined by the famous 19th century pathologist Virchow who attributed its major constituent to carbohydrate accumulation [ 1 ]. [dermatology-s10.cdlib.org]

  • Joint Effusion

    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 [neuromuscular.wustl.edu]

  • Pruritus

    Typically, in both pedigrees, the onset of symptoms was around puberty, and pruritus usually began on the lower legs. [ncbi.nlm.nih.gov]

    Ancillary Article Information DOI 10.1111/j.1600-0625.2010.01083.x Format Available Full text: HTML PDF Request Permissions Keywords apoptosis; gene expression; interleukin 31; oncostatin M; pruritus Publication History Issue online: 12 April 2010 Version [onlinelibrary.wiley.com]

    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]

    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]

    […] in pruritus, pigmentation or flattening of papules was labeled as 'no response'. [idoj.in]

  • Skin Lesion

    In primary systemic amyloidosis, skin lesions typically occur on the: A. Upper back B. Pretibial area C. Tongue D. Eyelids E. No skin lesion 2. In secondary systemic amyloidosis, skin lesions typically occur on the: A. Upper back B. [woundsresearch.com]

    Three family members had the characteristic skin lesion and also carried the gene for MEN 2a; two additional members carried the gene for MEN 2a, but did not manifest the observable skin changes associated with lichen amyloidosis. [annals.org]

    Silverstein SR: Primary, systemic amyloidosis and the dermatologist: where classic skin lesions may provide the clue for early diagnosis, Dermatol Online J 11:5, 2005. [clinicalgate.com]

    In lichen amyloidosis, PUVA has been reported to be effective in alleviating itch and flattening of skin lesions. [clinicaldermatology.eu]

    The skin lesions were more pronounced on the lower legs (Figure 1A ), back and waist (Figure 1B, 1C ), and the larger hypopigmented lesions were seen mainly on the lower legs. [bmcdermatol.biomedcentral.com]

  • Lichenification

    Abstract Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal dominant disorder associated with chronic itching and skin lichenification. [ncbi.nlm.nih.gov]

    Tanaka's study [ 16 ] demonstrated that most changes of gene expression revealed alterations in epidermal differentiation and proliferation consistent with lichenification. [bmcdermatol.biomedcentral.com]

    Wong reported the role of chronic friction in cutaneous amyloidosis. [1] Scratching has been known to produce traumatic and pressure changes such as excoriation, inflammation, pigmentation, and finally lichenification in the human skin. [idoj.in]

  • Follicular Plugging

    In both patients punch biopsies from the conchal bowl yielded a pauci-inflammatory specimen with orthohyperkeratosis and acanthosis (Fig. 4), as well as prominent follicular plugging (Fig. 5). [dermatology-s10.cdlib.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]

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 Skin [ingentaconnect.com]

Although most cases occur sporadically in people with no family history of the condition, there are rare familial forms caused by changes ( mutations ) in the OSMR or IL31RA gene. [3] [4] Treatment is based on the signs and symptoms present in each person [rarediseases.info.nih.gov]

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]

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]

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]

Whether there exists an association with regard to female sex hormones in the etiology of PCA still requires elaboration. [pigmentinternational.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]

Relevant External Links for OSMR Genetic Association Database (GAD) OSMR Human Genome Epidemiology (HuGE) Navigator OSMR Atlas of Genetics and Cytogenetics in Oncology and Haematology: OSMR No data available for Genatlas for OSMR Gene Novel IL31RA gene [genecards.org]

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]

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]

Sub track Toxin interactions and Symptoms Causes and Pathophysiology Diagnosis and Prevention Hygiene Hypothesis Liposuction Dermatology Conferences Cosmetology Conferences Plastic Surgery Conferences Dermatology Meetings Dermatologist Conferences 2019 [clinicaldermatology.euroscicon.com]

Etiology Pathophysiology The amyloid deposits in macular amyloidosis are keratinocyte-derived. [infectiousdiseaseadvisor.com]

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 Toxin interactions and Symptoms Causes and Pathophysiology Diagnosis and Prevention Hygiene Hypothesis Liposuction Dermatology Conferences Cosmetology Conferences Plastic Surgery Conferences Dermatology Meetings Dermatologist Conferences 2019 [clinicaldermatology.euroscicon.com]

The goal of treatment is to prevent secondary infection and to allow the lesions to heal on their own. [bobbybukamd.com]

Images