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{{DiseaseDisorder infobox |
  Name        = Porphyria cutanea tarda |
  ICD10      = {{ICD10|E|80|1|e|70}} |
  ICD9        = {{ICD9|277.1}} |
}}
{{SI}}
{{CMG}}; {{AE}} {{KS}}
{{CMG}}; {{AE}} {{KS}}




==Overview==
==Overview==
{{DiseaseDisorder infobox |
'''Porphyria cutanea tarda''' (PCT) is the most common subtype of [[porphyria]]. The disorder results from low levels of the [[enzyme]] responsible for the [[uroporphyrinogen III decarboxylase|fifth step]] in [[heme]] production. Heme is a vital molecule for all of the body's organs. It is a component of [[hemoglobin]], the molecule that carries [[oxygen]] in the blood.
  Name          = Pellagra |
  Image          = Pellagra NIH.jpg |
  Caption        = Pellagra sufferer with [[skin lesion]]s|
  ICD10          = {{ICD10|E|52||e|50}} |
  ICD9          = {{ICD9|265.2}} |
  ICDO          = |
  OMIM          = |
  DiseasesDB    = 9730 |
  MedlinePlus    = 000342 |
  eMedicineSubj  = ped |
  eMedicineTopic = 1755 |
  MeshName      = Pellagra |
  MeshNumber    = C18.654.521.500.133.699.529 |
}}
{{Search infobox}}
'''Pellagra''' is a [[vitamin]] [[deficiency disease]] caused by dietary lack of [[niacin]] (vitamin B3) and [[protein]], especially proteins containing the [[essential amino acid]] [[tryptophan]].<ref name=Pitche_2005>{{cite journal |author=Pitche P |title=Pellagra |journal=Sante |volume=15 |issue=3 |pages=205-8 |year=2005 |pmid = 16207585}}</ref> Because tryptophan can be [[Niacin#Biosynthesis|converted into niacin]], foods with tryptophan but without niacin, such as [[milk]], prevent pellagra. However, if dietary tryptophan is diverted into protein production, niacin deficiency may still result.  


Tryptophan is an [[essential amino acid]] found in meat, poultry, fish, and eggs. If your diet contains these foods, your need for niacin from other sources will be reduced.<ref>{{cite web | author = Haas EM | title = Vitamin B3&mdash;Niacin | work = Excepted from: Staying Healthy with Nutrition: The Complete Guide to Diet and Nutritional Medicine| url = http://www.healthy.net/scr/article.asp?ID=2125 | accessdate = 2007-06-18}}</ref>
==Presentation==
When signs and symptoms occur, they usually begin in adulthood and result from the skin becoming overly sensitive to sunlight. Areas of skin exposed to the sun develop severe blistering, scarring, changes in pigmentation, and increased hair growth. Exposed skin becomes fragile and is easily damaged. People with porphyria cutanea tarda also have increased iron levels in the liver. They face a higher risk of developing abnormal liver function and liver cancer.<ref>{{cite journal | author=Fracanzani AL, Taioli E, Sampietro M, Fatta E, Bertelli C, Fiorelli G, Fargion S | title=Liver cancer risk is increased in patients with porphyria cutanea tarda in comparison to matched control patients with chronic liver disease | journal=J Hepatol | year=2001 | pages=498-503 | volume=35 | issue=4  | id=PMID 11682034}}</ref>


The relationship between [[lysine]] and pellagra is unclear.<ref name=Bapurao_1978>{{cite journal |author=Bapurao S, Krishnaswamy K |title=Vitamin B6 nutritional status of pellagrins and their leucine tolerance |journal=Am J Clin Nutr |volume=31 |issue=5 |pages=819-24 |year=1978 |pmid = 206127}}</ref>
==Causes==
 
*In the acquired form, the signs and symptoms of this condition are triggered by non genetic factors such as alcohol abuse, excess iron, certain hormones, and viral infections. However, in the inherited form of the disease, which affects up to 20% of PCT patients, the condition arises from a mutation in the uroporphyrinogen decarboxylase gene, although environmental and chemical factors may trigger or exacerbate symptoms.
===Causes===
*Drug side effect: [[Chlorpropamide]], [[Tolbutamide]], [[Tolazamide]]
 
===Drug Causes===
 
* [[Isoniazid]]
 
==Symptoms==
The symptoms of pellagra include:
* High [[Photodermatitis|sensitivity to sunlight]]
* [[Aggression]]
* [[Dermatitis]]
* Smooth, beefy red [[glossitis]]
* Red [[skin lesion]]s
* [[Insomnia]]
* [[Weakness]]
* [[Mental confusion]]
* [[Diarrhea]]
* Eventually [[dementia]]
 
The main results of pellagra can easily be remembered as "the four D's": diarrhea, dermatitis, dementia, and death.<ref name=Hegyi_2004>{{cite journal |author=Hegyi J, Schwartz R, Hegyi V |title=Pellagra: dermatitis, dementia, and diarrhea |journal=Int J Dermatol |volume=43 |issue=1 |pages=1-5 |year=2004 |pmid = 14693013}}</ref>


==Diagnosis==
==Diagnosis==
===Physical Examination===
===Physical Examination===
====Skin====
====Skin====
=====Neck=====
=====Extremities=====
<gallery>
<gallery>


Image:Pellagra02.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda01.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda02.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




Image:Porphyria cutanea tarda03.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra03.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




Image:Porphyria cutanea tarda04.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra12.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra13.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>






Image:Pellagra36.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda05.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>








Image:Porphyria cutanea tarda06.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra47.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




Image:Pellagra48.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra25.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Porphyria cutanea tarda07.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra33.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
</gallery>


=====Face=====
Image:Porphyria cutanea tarda08.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
<gallery>


Image:Pellagra19.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




Image:Pellagra32.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda09.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra39.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


</gallery>


=====Trunk=====
Image:Porphyria cutanea tarda11.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
<gallery>


Image:Pellagra23.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra24.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra43.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra45.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda12.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


Image:Pellagra46.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


</gallery>


=====Extremity=====
<gallery>


Image:Pellagra04.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda13.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>






Image:Pellagra05.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda14.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




</gallery>


Image:Pellagra06.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
=====Face=====
<gallery>




Image:Pellagra07.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Image:Porphyria cutanea tarda10.jpg|Porphyria cutanea tarda. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>




</gallery>


Image:Pellagra08.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
==Epidemiology==
 
This type of porphyria occurs in an estimated 1 in 25,000 people, including both inherited and sporadic (noninherited) cases. An estimated 80 % of porphyria cutanea tarda cases are sporadic. The exact frequency is not clear because many people with the condition never experience symptoms.
 
Image:Pellagra09.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra10.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra11.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra14.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


==Genetics==


Image:Pellagra15.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Inherited mutations in the ''[[UROD]]'' [[gene]] cause about 20 % of cases (the other 80 % of cases do not have [[mutation]]s in UROD, and are classified as sporadic). UROD makes an enzyme called [[uroporphyrinogen III decarboxylase]], which is critical to the chemical process that leads to heme production. The activity of this enzyme is usually reduced by 50 % in all tissues in people with the inherited form of the condition.  


Nongenetic factors such as [[alcohol abuse]], excess [[iron]], and others listed above can increase the demand for heme and the enzymes required to make heme. The combination of this increased demand and reduced activity of uroporphyrinogen decarboxylase disrupts heme production and allows byproducts of the process to accumulate in the body, triggering the signs and symptoms of porphyria cutanea tarda.


The ''[[HFE]]'' gene makes a protein that helps cells regulate the absorption of iron from the digestive tract and into the cells of the body. Certain mutations in the ''HFE'' gene cause [[hemochromatosis]] (an [[iron overload disorder]]). People who have these mutations are also at an increased risk of developing porphyria cutanea tarda.


Image:Pellagra16.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
In the 20% of cases where porphyria cutanea tarda is inherited, it is inherited in an [[autosomal dominant]] pattern, which means one copy of the altered gene is sufficient to decrease enzyme activity and cause the signs and symptoms of the disorder.


[[Image:autodominant.jpg|thumb|center|20% of cases of porphyria cutanea tarda are inherited in an [[autosomal dominant]] pattern.]]


==Treatment==
[[Chloroquine]] and [[venesection]] can be part of a management strategy.<ref>{{cite journal | author=Sarkany RP | title=The management of porphyria cutanea tarda | journal=Clin Exp Dermatol | year=2001 | pages=225-32 | volume=26 | issue=3  | id=PMID 11422163}}</ref>


 
==Cultural references==
Image:Pellagra17.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
Porphyria Cutanea Tarda is also the name of a song by the punk band AFI on their album Black Sails in the Sunset.
 
 
 
Image:Pellagra18.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra20.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra21.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra22.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
Image:Pellagra26.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra27.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra28.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra29.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra30.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra34.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra35.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra37.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra38.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra40.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra41.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra42.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra44.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra49.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra50.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra51.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra53.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra54.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra55.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra56.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra57.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra58.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra59.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra60.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra61.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra62.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra63.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Pellagra64.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Pellagra65.jpg|Pellagra. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
</gallery>
 
==Epidemiology==
Pellagra can be common in people who obtain most of their [[food energy]] from [[maize]], since untreated corn is a poor source of [[niacin]] (vitamin B3). Corn is also a [[Essential amino acid#Use of essential amino acids|poor source of tryptophan]].  This disease can be common among people who live in rural South America where corn is a staple. The symptoms usually appear during spring, increase in the summer due to greater sun exposure, and return the following spring. It is also one of several diseases of malnutrition common in Africa, and was [[endemic (epidemiology)|endemic]] in the poorer states of the U.S. south like Mississippi and Alabama as well as among the inmates of jails and orphanages, where it was studied by [[Joseph Goldberger]] who conducted experiments in the penal colony in Rankin. Alkali treatment of the corn corrects the niacin deficiency, and was a common practice in native American cultures that grew corn. The amino acid deficiency must be balanced by consumption of other sources of protein. It was also common amongst prisoners of Soviet labor camps, the infamous Gulag. Also found in cases of chronic alcoholism.
 
==Prognosis==
Untreated, the disease can kill within four or five years.
 
Pellagra can be treated by treatment with niacin (usually as niacinamide). The frequency and amount of niacinamide administered depends on the degree to which the condition has progressed.
 
==History==
[[Image:Joseph Goldberger 01.jpg|thumb|100px|left|Portrait of Dr. Joseph Goldberger]]
The traditional food preparation method of corn, [[nixtamalization]], by native New World cultivators, who had domesticated corn, required treatment of the grain with [[calcium hydroxide|lime]], an [[alkali]]. It has now been shown that the lime treatment makes niacin nutritionally available and reduces the chance of developing pellagra. When corn cultivation was adopted worldwide, this preparation method was not accepted because the benefit was not understood. The original cultivators, often heavily dependent on corn, did not suffer from pellagra. Pellagra became common only when corn became a staple that was eaten without the traditional treatment.
 
Pellagra was first described in Spain in 1735 by Gaspar Casal, who published a first clinical description in his posthumous "Natural and Medical History of the Asturian Pricipality" (1762). This led to the disease being knownn as "Asturian leprosy", and it is recognised as the first modern pathological description of a syndrome(1).  It was an endemic disease in northern Italy, where it was  named "pelle agra" (pelle = skin; agra = rough) by Francesco Frapoli of Milan.<ref>{{cite web | title = Definition of Pellagra| work = MedicineNet.com | url = http://www.medterms.com/script/main/art.asp?articlekey=4821 | accessdate = 2007-06-18}}</ref>  Because pellagra outbreaks occurred in regions where [[maize]] was a dominant food crop, the belief for centuries was that the maize either carried a toxic substance or was a carrier of disease. It was not until later that the lack of pellagra outbreaks in Mesoamerica, where maize is a major food crop (and is processed), that the idea was considered that the causes of pellagra may be due to factors other than toxins.
 
In the early 1900s, pellagra reached epidemic proportions in the American South. There were 1,306 reported pellagra deaths in South Carolina during the first ten months of 1915; 100,000 Southerners were affected in 1916. At this time, the scientific community held that pellagra was probably caused by a [[germ]] or some unknown [[toxin]] in corn.<ref name=Bollet_1992>{{cite journal |author=Bollet A |title=Politics and pellagra: the epidemic of pellagra in the U.S. in the early twentieth century |journal=Yale J Biol Med |volume=65 |issue=3 |pages=211-21 |year= 1992|pmid = 1285449}}</ref> The Spartanburg Pellagra Hospital in Spartanburg, South Carolina, was the nation's first facility dedicated to discovering the cause of pellagra. It was established in 1914 with a special congressional appropriation to the U.S. Public Health Service (PHS) and set up primarily for research.  In 1915 [[Joseph Goldberger]], assigned to study pellagra by the [[Surgeon General of the United States]], showed that pellagra was linked to diet by inducing the disease in prisoners, using the Spartanburg Pellagra Hospital as his clinic. By 1926, Goldberger established that a balanced diet or a small amount of baker's yeast prevented pellagra. Skepticism still persisted in the medical community until  1937 when [[Conrad Elvehjem]] showed that the vitamin niacin cured pellagra (manifested as [[Black hairy tongue|black tongue]]) in dogs. Later studies by Tom Spies, Marion Blankenhorn and Clark Cooper established that niacin also cured pellagra in humans, for which ''Time'' Magazine dubbed them its 1938 Men of the Year in comprehensive science.


==References==
==References==
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{{Reflist|2}}
 
===Other reading===
(1) Stratigos JD, Katsambas A. Pellagra: a still existing disease. B J
Dermatol 1977; 96: 99-106.
<div class="references-small" style="-moz-column-count:2; column-count:2;">
* {{cite journal | author = Hampl JS, Hampl WS. | title = Pellagra and the origin of a myth: evidence from European literature and folklore. | journal = J Roy Soc Med. | year = 1997 | volume = 90 | issue = | pages = 636&ndash;639 | url= http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1296679}}
* {{cite journal | author = | title =  Reports and Resolutions of the General Assembly of the State of South Carolina, Regular Session Commencing January 11, 1916. | journal = Annual Report of the State Board of Health (1915-1916). | year = 1916 | location = Columbia, S.C. | volume = 4 | publisher = Gonzales and Bryan, state printers | url = }}
* {{cite book |author=Beardsley E | title=The Spartanburg Pellagra Hospital. In: The South Carolina Encyclopedia |publisher=University of South Carolina Press |location=Columbia, S.C |year=2006 |pages= |isbn=1-57003-598-9}}
</div>


==External links==
==Additional Resources==
* {{DermNet|systemic/pellagra}}
* {{cite journal | author=Kauppinen R | title=Porphyrias | journal=Lancet | year=2005 | pages=241-52 | volume=365 | issue=9455  | id=PMID 15652607}}
* [http://www.cyberparent.com/alternativehealth/pellagra.htm cyberparent.com]
* {{cite journal | author=Lecha M, Herrero C, Ozalla D | title=Diagnosis and treatment of the hepatic porphyrias | journal=Dermatol Ther | year=2003 | pages=65-72 | volume=16 | issue=1  | id=PMID 12919129}}
* [http://www.wholegrain.umn.edu/grains/corn.cfm wholegrain.umn.edu]
* {{cite journal | author=Nordmann Y, Puy H | title=Human hereditary hepatic porphyrias | journal=Clin Chim Acta | year=2002 | pages=17-37 | volume=325 | issue=1-2  | id=PMID 12367763}}
* {{cite journal | author=Sassa S | title=The porphyrias | journal=Photodermatol Photoimmunol Photomed | year=2002 | pages=56-67 | volume=18 | issue=2  | id=PMID 12147038}}
* [http://web.uct.ac.za/depts/porphyria/professional/prof-pct.htm "Porphyria Cutanea Tarda"] Porphyria South Africa, University of Cape Town/Groote Schurr Hospital




{{Nutritional pathology}}
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{{Endocrine, nutritional and metabolic pathology}}
{{SIB}}


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[[da:Pellagra]]
[[Category:Porphyrias]]
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]


Overview

Porphyria cutanea tarda (PCT) is the most common subtype of porphyria. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin, the molecule that carries oxygen in the blood.

Presentation

When signs and symptoms occur, they usually begin in adulthood and result from the skin becoming overly sensitive to sunlight. Areas of skin exposed to the sun develop severe blistering, scarring, changes in pigmentation, and increased hair growth. Exposed skin becomes fragile and is easily damaged. People with porphyria cutanea tarda also have increased iron levels in the liver. They face a higher risk of developing abnormal liver function and liver cancer.[1]

Causes

  • In the acquired form, the signs and symptoms of this condition are triggered by non genetic factors such as alcohol abuse, excess iron, certain hormones, and viral infections. However, in the inherited form of the disease, which affects up to 20% of PCT patients, the condition arises from a mutation in the uroporphyrinogen decarboxylase gene, although environmental and chemical factors may trigger or exacerbate symptoms.
  • Drug side effect: Chlorpropamide, Tolbutamide, Tolazamide

Diagnosis

Physical Examination

Skin

Extremities
Face

Epidemiology

This type of porphyria occurs in an estimated 1 in 25,000 people, including both inherited and sporadic (noninherited) cases. An estimated 80 % of porphyria cutanea tarda cases are sporadic. The exact frequency is not clear because many people with the condition never experience symptoms.

Genetics

Inherited mutations in the UROD gene cause about 20 % of cases (the other 80 % of cases do not have mutations in UROD, and are classified as sporadic). UROD makes an enzyme called uroporphyrinogen III decarboxylase, which is critical to the chemical process that leads to heme production. The activity of this enzyme is usually reduced by 50 % in all tissues in people with the inherited form of the condition.

Nongenetic factors such as alcohol abuse, excess iron, and others listed above can increase the demand for heme and the enzymes required to make heme. The combination of this increased demand and reduced activity of uroporphyrinogen decarboxylase disrupts heme production and allows byproducts of the process to accumulate in the body, triggering the signs and symptoms of porphyria cutanea tarda.

The HFE gene makes a protein that helps cells regulate the absorption of iron from the digestive tract and into the cells of the body. Certain mutations in the HFE gene cause hemochromatosis (an iron overload disorder). People who have these mutations are also at an increased risk of developing porphyria cutanea tarda.

In the 20% of cases where porphyria cutanea tarda is inherited, it is inherited in an autosomal dominant pattern, which means one copy of the altered gene is sufficient to decrease enzyme activity and cause the signs and symptoms of the disorder.

20% of cases of porphyria cutanea tarda are inherited in an autosomal dominant pattern.

Treatment

Chloroquine and venesection can be part of a management strategy.[3]

Cultural references

Porphyria Cutanea Tarda is also the name of a song by the punk band AFI on their album Black Sails in the Sunset.

References

  1. Fracanzani AL, Taioli E, Sampietro M, Fatta E, Bertelli C, Fiorelli G, Fargion S (2001). "Liver cancer risk is increased in patients with porphyria cutanea tarda in comparison to matched control patients with chronic liver disease". J Hepatol. 35 (4): 498–503. PMID 11682034.
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 "Dermatology Atlas".
  3. Sarkany RP (2001). "The management of porphyria cutanea tarda". Clin Exp Dermatol. 26 (3): 225–32. PMID 11422163.

Additional Resources

  • Kauppinen R (2005). "Porphyrias". Lancet. 365 (9455): 241–52. PMID 15652607.
  • Lecha M, Herrero C, Ozalla D (2003). "Diagnosis and treatment of the hepatic porphyrias". Dermatol Ther. 16 (1): 65–72. PMID 12919129.
  • Nordmann Y, Puy H (2002). "Human hereditary hepatic porphyrias". Clin Chim Acta. 325 (1–2): 17–37. PMID 12367763.
  • Sassa S (2002). "The porphyrias". Photodermatol Photoimmunol Photomed. 18 (2): 56–67. PMID 12147038.
  • "Porphyria Cutanea Tarda" Porphyria South Africa, University of Cape Town/Groote Schurr Hospital


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