WHAT IS DEGOS DISEASE ?

 DEGOS DISEASE 

Degos disease ,also known as kohlmeier degos disease or malignant atrophic papulosis , is an extremely rare  condition caused by blockage  of arteries and veins . Individuals with  this condition will develop papules. Those diagnosed with this disease may also  develop  complication due to impairment of internal  organs . There are fewer than 50  living patients presently known worldwide , and fewer than 200 reported in medical literature . 

Many  individuals may go  undiagnosed due to rarity of the disease . Most individual  develop symptoms  between  the  ages  of 20 -50 , however cases outside of this age range have been reported as well. Degos disease also  know as malignant atrophic papulosis is characterized by vasculopathy of unknown origin  origin and independently described by Degos in 1942 . 

DEGOS DISEASE PICTURES :







WHAT ARE THE SIGNS AND SYMPTOMS OF DEGOS DISEASE ?

The characteristics  symptoms of degos disease  in the development  of papules . Individual  may have skin lesions or rashes , but they will proceed to develop  distinct  bumps or papules. Papules are circular in shape , have a porcelain - white  center and red border. The white centers will skin in and only the border  will remain raised . Papules appear on thr trunk  and upper extremities and are not found on the individuals 's palms , soles, scalp , or face . 
   
     Symptoms vary  depending on whether an individual has the benign  variant  or malignant  variant of the disease .  Both the benign and malignat forms have development of the characteristic papules .  Individuals  develops the maligant form , it means  that not only are  the papules persisting  anywhere from  a few years to throughout  thier whole lives . In the benign form , no inner organs  are affected . Most maligant cases involve  problems of the gastrointestinal tract leading  to small intestine  lesions ,  abdominal pain , diarrhea , and bowel  perforation . Symptoms  that may develop  from damage  to these organs  include  double vision , clouding of lenses of eyes . swelling  of the  optic disc , partial loss of vision , shortness of breath , chest psin , and thickening  of pericardium . 
Someone with the benign form may suddenly develop  symptoms of  the malignant form . Symptoms can last anywhere  from a few  weeks to several  years . Onset of symptoms  typically begins to manifest  between the ages of 20- 50 . A few  cases of this condition  in newborn . 



CAUSES OF DEGOS DISEASE :

The papules characteristics for this disease develop due to  infarctions , or blockage in small - medium  arteries and veins . The  underlying  cause is unknown  for this  disease . Some cases have shown signs of inheritance between first -degree  relatives . it has been suggested that the disease has a familial inheritance pattern , it is thougt to be an autosomal dominant disorder , in most cases of familial inheritance , the benign variant of the disease has been  present . 
         Due to the lack of  knowledge of the pathomechanism for this  condition prevention  strategies  are not known . However to prevent worsening of symptoms , consistents  evalutions should be conducted  by a physician .


HOW TO DIAGONSIS OF DEGOS DISEASE ?

Individuals may be diagonsed  with the benign form if only  the  papules are present . however an individuals  may be diagonsed with the malignant  form  if involvement of other  organs  like the lungs ,  intestine and or central  nervous system  occurs . The maligant , or systematic  form of this condition may suddenly  develop  even after having papules present for several years ,Order to quickly  diagnose this shift to the malignant  variant  of the disease , it is important  for individuals  to have  consistent  follow up  evalution . In these evaltion , depending  on which  organs are suspected to be involved , the following procedures  and  tests  may be conducted : skin  inspection , brain magnetic  resonance  tomography , colonoscopy , chest X- ray and abdominal ultrasound . 

Clinical evalution and identification of characteristics papules  may allow a dermatologist  to  diagnose degos disease . The papules have a white center and are bordered with a red ring .  After  lesions  begin to appear  , the diagnose for Degos disease can be supported by histological  findings . This shape is due to the blockage of small arteries . 

HOW TO TREAT DEGOS DISEASE ?

 Due to the lack of knowledge around the underlying  mechanism of malignant  atrophic papulosis , an effective  treatment method has not been developed . Treatments  for this condition  is symptomatic . Several treatments  methods  have been tested and are still being  developed as more information regarding the condition is  found . 
    After  treating conditions comoroid with Degos disease , physicians have recently found improvement in symptoms with  the use of eculizumab and treprostinil . Treprotinil use has been  reported  to results  in clearing of gastrointestinal and central nervous system finding as well, as  clearing of cutaneous lesions , but reports are limited . It may also increase the population of circulating endothelial cells, allowing angiogenesis . 

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