Degos Disease and the SSA's Compassionate Allowance
Compassionate Allowance Series By Stephanie O. Joy, Esq., JoyDisability.com
7/30/2021
Degos Disease is a recognized Compassionate Allowance disability by the Social Security Administration. https://secure.ssa.gov/poms.nsf/lnx/0423022380
Background
Degos Disease is also known as Degos-Kohlmeier Disease; Degos Syndrome; Malignant Atrophic Papulosis; MAP; Kohlmeier disease; Papulosis Atrophican Maligna.
Degos Disease is an SSA Compassionate Allowance disease and if specific Medical Evidence (from medical records) may be developed and presented, a person with Degos Disease may be able to more quickly file for and prevail on a claim for SSDIB or SSI. (Scroll below for details on what the SSA suggests be provided and evaluated to determine if a Compassionate Allowance should be determined for Degos Disease for a particular claimant.) https://secure.ssa.gov/poms.nsf/lnx/0423022380
Background of Degos Disease This Compassionate Allowance disease is a disease of the blood vessels that is rare and non-inflammatory. Degos Disease is recognized as a narrowing and blockage in small and medium sized arteries. The organ system it attacks suffers from ischemia and tissue infarction. It visually manifests itself with skin(s) lesions. It may affect blood vessels which, liver, skin, GI tracts, kidneys and even the central nervous system. It is thought that the propeller of the development of Degos Disease may be Immune System Dysregulation.
There are generally two variants to Degos Disease progression. The first involves skin lesions. Their duration may vary and last as short as a few weeks or as long as a number of years. They are visible red sores that leave scars with white centers. The skin lesions may become chronic, and may include ocular lesions, ischemia of the bowel, spinal legions and inflammation of several separate nerves (known as mononeuritis multiplex). Affects may include cognitive difficulties, headaches and seizures (epilepsy). In the second phase The second stage Degos Disease includes internal lesions of organs. These lesions can become result in bowel ischemia, chronic skin lesions, ocular lesions, strokes, spinal lesions, mononeuritis multiplex (inflammation of several separate nerves), epilepsy, headaches, or cognitive disorders. In the second stage, which is more fatal and multi-organ, one may experience weight loss, diarrhea, abdominal pain. If the lesions are intestinal, the can perforate the bowel, leading to sepsis, a very life threatening condition. At this second stage, there may be a two to three year fatality time frame.
Demographics
The disease is considered a young adult’s disease and usually affects men, but not always. There is no known cause, cure or treatment to slow its progression remains unavailable. Treatment is directed toward the specific symptoms of each patient. https://rarediseases.org/rare-diseases/degos-disease/#causes
Prognosis
Despite the above, the prognosis for any particular person may depend on the extent of blood vessel involvement. Those with isolated skin macules may have a good prognosis, while those with multisystem disease would more likely face the life threatening complications described in the literature. https://rarediseases.info.nih.gov/diseases/6249/malignant-atrophic-papulosis
Medications
Although there are no curative or treatment drugs to halt or slow down the disease, there are drugs prescribed to address symptoms. Drugs that inhibit the activity of platelets, known as anti-platelet or anti-coagulation drugs may be used. Aspirin and Dipyridamole combined has been reported to improve skin and eye lesions in two individuals who did not have systemic involvement.
SSA handling of Degos Disease claims The SSA describes what it finds to be proper diagnostic reporting for a consideration as a Compassionate Allowance.
Physical examination;
Identification of characteristic findings (e.g. skin lesions);
CT/ MRI scan; and
Microscopic examination of affected skin tissue that reveals distinctive changes to the tissue.
The medical evidence expected to be found in a claimant’s submitted claim includes:
Clinical history and examination that describes the diagnostic features of the impairment;
Dermatology consultation that evaluates the ability of the individual to adjust to the reduced oxygen carrying capacity of blood;
Laboratory tests measuring blood composition; and
MRI/CT scans.
The SSA Bluebook Listings that an attorney may urge the SSA to consider include 14.03
and 114.03. These may suffice if the MER (medical evidence of record) demonstrates that the claim equals these listings, as there is no avenue for actually “meeting” those particular listings, due to the currently known nature of Degos Disease.
Current Clinical Trials
Information on current clinical trials is posted on the Internet at http://www.clinicaltrials.gov. "All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site."
2018 Right to Try Law
In 2018, President Trump signed into law the Right to Try law. Several states had not yet allowed patients to choose to treat with what was available but not yet fully approved by FDA as a treatment, and many with rarer diseases are not viable candidates for clinical trials. This federal law leveled the playing field for all – without the need to uproot patient and family to a different State recognizing need for a Right to Try, during such a life-threatening situation.
While this information on Degos Disease does not extend to whether treatments passing the Right to Try law may be used for Degos Disease, (fluid area of law and medicine), this law provides incentive for research and manufacturing of treatments for such rare diseases that would otherwise be ignored due to lack of profit for those companies that have the means to discover a viable treatment for Degos Disease and many other rarer diseases. This law provides life-saving rights for many to choose to take informed action to help save themselves. We applaud.
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If your or someone know is or may be suffering from Degos Disease and you want to discuss helping them prove and prevail on a Social Security Disability claim (or SSI if needed), I welcome you to contact me at stephaniejoy@joydisability.com, or go to http://joydisability.com and fill out the quick and simply evaluation form and I will call you at the contact number you provide.
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