Early Palliative Care in Degos Disease: A Case for Proactive Support in Rare, Life-Limiting Illnesses

Kohlmeier-Degos disease is one of the most rarely noted diseases in the medical literature. Due to its rarity, many individuals are likely to go undiagnosed or to be diagnosed in more advanced stages. In those individuals with the disease, the initial presentation consists of characteristic skin lesions (porcelain-white macules). These lesions can remain limited to the skin only, which indicates benign disease. Unfortunately, some patients will go on to develop systemic disease, which is life-threatening and can form lesions in the gastrointestinal tract, central nervous system, heart, and lungs. Because of the complexity and aggressive nature of this disease, early palliative medicine involvement could prove beneficial to support patients with symptom management as well as to support patients and their families in navigating difficult clinical decision-making while pursuing treatment. To the best of our knowledge, this is among the first case reports examining the potential benefits of early integration of palliative medicine and its role in not only systemic Degos disease, but also other rare life-limiting illnesses. This case study will highlight the role of the palliative medicine team's involvement and the importance of a multidisciplinary team in treating those with rare life-threatening diseases.