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Encyclopedia > Acrodermatitis enteropathica
Acrodermatitis enteropathica
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ICD-10
ICD-9
ICD-O:
OMIM 201100
DiseasesDB [1]
MedlinePlus [2]
eMedicine derm/5

Acrodermatitis enteropathica is an autosomal recessive disorder characterized by periorificial (around the natural orifices) and acral (in the limbs) dermatitis, alopecia (loss of hair), and diarrhea. Similar features may be present in acquired zinc deficiency. This disease also is related to deficiency of zinc due to congenital causes. Some synonyms of this condition are: The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... An autosome is a non-sex chromosome. ... In genetics, the term recessive gene refers to an allele that causes a phenotype (visible or detectable characteristic) that is only seen in a homozygous genotype (an organism that has two copies of the same allele). ... Dermatitis is a term literally meaning inflammation of the skin. It is usually used to refer to eczema, which is also known as Dermatitis eczema. ... Alopecia is a set of disorders ranging from male and female pattern alopecia (alopecia androgenetica), to alopecia areata, which involves the loss of some of the hair from the head, alopecia totalis, which involves the loss of all head hair, to the most extreme form, alopecia universalis, which involves the... Diarrhea (American English) or diarrhoea (Commonwealth English) is a condition in which the sufferer has frequent and watery, chunky, or loose bowel movements (from the ancient Greek word διαρροή = leakage; lit. ... General Name, Symbol, Number zinc, Zn, 30 Chemical series transition metals Group, Period, Block 12, 4, d Appearance bluish pale gray Atomic mass 65. ...

  • Brandt Syndrome
  • Danbolt-Cross Syndrome
  • Congenital Zinc Deficiency

Contents


Pathogenesis

The genetic mutation of SLC39A4 gene on Chromosome 8q locus 24.3 appears to be the cause. The SLC39A4 gene appears to encode a transmembrane protein that serves as a zinc uptake protein. The features of the disease usually start manifesting as an infant is weaned from breast milk. This has led some scientists to suspect that human milk contains a beneficial substance that helps uptake of zinc and prevents the disease from being manifested while an infant is on breast milk. In biology, mutations are changes to the genetic material (usually DNA or RNA). ... The word locus (plural loci) is Latin for place: In biology and evolutionary computation, a locus is the position of a gene (or other significant sequence) on a chromosome. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... General Name, Symbol, Number zinc, Zn, 30 Chemical series transition metals Group, Period, Block 12, 4, d Appearance bluish pale gray Atomic mass 65. ...


Presentation

Features of acrodermatitis enteropathica start appearing in the first few months of life, as the infant discontinues breast milk. There are erythematous patches and plaques of dry, scaly skin. The lesions may appear eczematous, or may evolve further into crusted vesicles , bullas or pustules. The lesions are frequent around natural orifices like the mouth (peri-oral) and anus (peri-anal), and also in hands, feet and scalp. There may be suppurative inflammation of the nail fold surrounding the nail plate - known as paronychia. Alopecia - loss of hair from scalp, eyebrows and eyelashes may occur. The skin lesions may be secondarily infected by bacteria such as Staphylococcus aureus or fungi like Candida albicans. These skin lesions are accompanied by diarrhea. Erythema is an abnormal redness of the skin caused by capillary congestion. ... A Patch can refer to several different things: A piece of fabric. ... The word plaque can have different connotations and can also be spelt placque: Look up Plaque on Wiktionary, the free dictionary Dental plaque is a yellowish biofilm that builds up on the teeth formed by bacteria embedded in a matrix of polymers of bacterial and salivary origin. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... In cell biology, a vesicle is a relatively small and enclosed compartment, separated from the cytosol by at least one lipid bilayer. ... A blister caused by an encounter with a muslim A blister or bulla is a defense mechanism of the human body. ... An abscess is a collection of pus collected in a cavity formed by the tissue on the basis of an infectious process (usually caused by bacteria or parasites) or other foreign materials (e. ... The scalp is the skin on the head from which head hair grows. ... Pus is a whitish-yellow or yellow substance that can be found in regions of bacterial infection, including superficial infections, such as pimples. ... Inflammation is the first response of the immune system to infection or irritation and may be referred to as the innate cascade. ... Paronychia is an often tender infection or inflammation around the base of the nail fold. ... Alopecia is a set of disorders ranging from male and female pattern alopecia (alopecia androgenetica), to alopecia areata, which involves the loss of some of the hair from the head, alopecia totalis, which involves the loss of all head hair, to the most extreme form, alopecia universalis, which involves the... Subgroups Actinobacteria Aquificae Bacteroidetes/Chlorobi Chlamydiae/Verrucomicrobia Chloroflexi Chrysiogenetes Cyanobacteria Deferribacteres Deinococcus-Thermus Dictyoglomi Fibrobacteres/Acidobacteria Firmicutes Fusobacteria Gemmatimonadetes Nitrospirae Planctomycetes Proteobacteria Spirochaetes Thermodesulfobacteria Thermomicrobia Thermotogae Bacteria (singular: bacterium) are a major group of living organisms. ... Binomial name Staphylococcus aureus Rosenbach, 1884 Staphylococcus aureus (which is occasionally given the nickname golden staph) is a bacterium, frequently living on the skin or in the nose of a healthy person, that can cause illnesses ranging from minor skin infections (such as pimples, boils, and cellulitis) and abscesses, to... Divisions Chytridiomycota Deuteromycota Zygomycota Glomeromycota Ascomycota Basidiomycota Fungus growing on a tree in Borneo A fungus (plural fungi) is a eukaryotic organism that digests its food externally and absorbs the nutrient molecules into its cells. ... Binomial name Candida albicans (C.P. Robin) Berkhout Candida albicans, a diploid sexual fungus (a form of yeast) is the causal agent of opportunistic infections in humans, the most common being oral and vaginal infections. ...


Treatment

Without treatment, the disease is fatal and affected individuals may die within a few years. However, there is no absolute treatment of the condition. Lifelong dietary zinc supplementation in the range of greater than 1-2 mg/kg of bodyweight per day is usually needed. General Name, Symbol, Number zinc, Zn, 30 Chemical series transition metals Group, Period, Block 12, 4, d Appearance bluish pale gray Atomic mass 65. ... Mg or mg or MG may stand for: machine gun Madagascar, ISO country code Malagasy language, in ISO 639 magnesium (Mg) MG (car), the car company milligram (mg) megagram (Mg) or Tonne MG stands for Minas Gerais, one of the states from Brazil Metal Gear or Metal Gear Solid This... KG, Kg or kg may indicate: A Kampfgeschwader, a bomber squadron of the former German Luftwaffe Basketball Player Kevin Garnett An abbreviation for kilogram (always kg) Knight of the Garter, a British decoration Kommanditgesellschaft, German version of a limited partnership Kongo language (ISO 639 alpha-2) An abbreviation for konig...


External links

Organizations and resources

The following organizations provide information and patient support resources:


  Results from FactBites:
 
Acrodermatitis Enteropathica (7075 words)
Acrodermatitis enteropathica-like eruption in an infant with nonketotic hyperglycinemia.
Acrodermatitis enteropathica is a rare inherited disorder characterized by zinc deficiency and a triad of dermatitis, diarrhea, and alopecia.
Acrodermatitis enteropathica is a rare autosomal recessive condition of zinc deficiency characterised by chronic diarrhoea associated with failure to thrive, periorificial dermatitis and alopecia, susceptibility to infections and behavioural changes.
Medical Dictionary: Acrodermatitis enteropathica - WrongDiagnosis.com - WrongDiagnosis.com (315 words)
Acrodermatitis enteropathica: A rare, chronic condition that occurs in infants and involves autosomal zinc malabsorption.
Acrodermatitis enteropathica: a severe human skin and gastrointestinal disease inherited as a recessive autosomal trait that is characterized by the symptoms of zinc deficiency and clears up when zinc is added to the diet.
Acrodermatitis enteropathica : severe human skin and gastrointestinal disease inherited as a recessive autosomal trait that is characterized by the symptoms of zinc deficiency and clears up when zinc is added to the diet.
  More results at FactBites »


 
 

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