INTRODUCTION: 

We report a case of Degos Disease, an extremely rare multisystem vasculopathy of unknown cause - only 200 cases have been described in the medical literature. 

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CASE HISTORY: 

A 26 year old male  from a small village in India, presented with 2 month history of difficulty walking and urinary incontinence. He denied any back pain. He was noted to have papules on his hands, feet and back but stated he had had them for more than 2 years. He denied any pain or itching on the papules.MRI of the lumbar spine revealed no abnormalities. The cerebrospinal fluid examination was within normal limits. MRI of the brain revealed hyperintensities in the left temporo-parietalregion that did not follow any vascular pattern. Vasculitis workup was negative but he was treated with high dose steroids for a possible autoimmune etiology but in spite of treatment, he became quadriparetic. During the hospital course, he complained of abdominal pain and developed an intestinal perforation. His intestines were found to have multiple tiny infarcts all over the bowel during surgery.  A repeat lumbar puncture revealed proteins of 705 with TLC of 300. Skin biopsy of the papules was sent at this point that revealed the diagnosis of Degos disease. As eculizimab was not available, he was treated with Rituximab. He received 2 doses of Rituximab and died of sepsis in the  3^rd week of hospitalization.

DISCUSSION:

Degos disease, also known as Kohlmeier-Degos disease or malignant atrophic papulosis(MAP)is an extremely rare condition, that causes widespread thrombosis, particularly of the vessels of the skin, central nervous system and gastrointestinal tract. Due to its early benign presentation, the diagnosis is often missed. Even with early treatment, survival is only 15%. 

A diagnosis of Degos Disease should be kept in mind when there is cutaneous, gastrointestinal and nervous system involvement.