Overview

Degos disease is a rare condition characterised by the occlusion (blockage or closing) of blood vessels. The disease primarily affects the skin and internal organs, such as the gastrointestinal tract, and can cause skin malformations and potential organ damage.

During pregnancy, blood volume increases to support the growth of a new life. As the body undergoes significant changes and each organ is required to work harder, pregnant women with Degos disease may experience unique challenges and uncertainties. 

What is degos disease?

Degos disease is a rare disease and is also called malignant atrophic papulosis. Less than 200 cases have been recorded worldwide, most of which are in individuals aged between 20 and 50 years old.¹

The disease can affect:

• The skin 

Common symptoms of Degos disease include skin lesions or rashes, which can develop into papules. They mostly appear on the torso and arms. 

• Internal organs

Degos can also affect internal organs (referred to as systemic degos). The majority of systemic Degos cases affect the gastrointestinal tract (73%), and the rest affect the central nervous system and multiple organ systems (64%, respectively).¹

The skin and internal organs are affected when blood vessels become damaged or blocked. The exact reason for the damage is unknown, however it is believed that it could result from inflammation of the blood vessels. Damaged or blocked blood vessels will have reduced ability to allow oxygen to pass through it (permeability), depriving the areas/organs the blood vessels lead to of oxygen. When the area affected is the skin, rashes can form papules. When the blood vessels do not supply oxygen to internal organs, there can be serious complications and even organ death. 

The symptoms of Degos disease depend on the affected organ, but can involve gastrointestinal tract bleeding, abdominal pain, and respiratory difficulties. 

Pregnancy and degos disease

Pregnancy causes a complete transformation in the mother’s body in order to accommodate a growing life. This causes several changes, including;

• Increased blood volume
• Higher risk of blood clotting
• Immune system shifts

These changes are normal and happen in order to grow human life and facilitate childbirth whilst minimising blood loss.²

In Degos disease, where blood vessels are already affected, these normal changes can complicate a pregnancy and deteriorate disease symptoms. 

Risks and complications

Dealing with Degos disease during pregnancy carries certain risks and complications for both mother and baby. 

Maternal symptoms include:

• Worsening of skin spots

The number and size of papules can increase during pregnancy because hormonal, immune, and blood-clotting changes increase the likelihood of blood vessel blockages that cause these leisons.³

• Higher chance of blood clots 

One of the major shifts the body undergoes during pregnancy is the blood clotting mechanism, which preventsexcessive bleeding during childbirth. In Degos disease, blood flow is already reduced in some areas, and in combination with a pregnancy, there is a risk of permanent vessel blockage, potentially worsening skin lesions and affecting body tissues.⁴

• Organ damage 

In systemic Degos disease, blockages can occur in the vessels supplying vital organs. If certain organs do not receive an adequate blood supply, organ damage can occur. 

Blockages in the gut can lead to tissue damage, bleeding and pain. If blockagesaffect the brain, it can lead to headaches, dizziness, confusion and weakness. 

The circulatory changes that occur in a pregnancy pose a greater risk for organ damage in women with Degos disease.⁵

Fetal symptoms include:

• Inadequate growth and nutrition 

If the affected blood vessels supply the placenta, reduced blood flow can deprive the baby of important nutrients required for growth.

• Preterm birth 

When the body suspects that the mother’s health is under strain, early labour may be initiated. Doctors might even recommend early delivery to avoid complications later on. Premature babies can face breathing or feeding difficulties and sometimes health complications that may persist later in life. 

• Fetal loss

In severe cases, Degos disease may entirely block blood vessels supplying the placenta. If oxygen and nutrients are not delivered to the baby, the pregnancy can result in a miscarriage or stillbirth.⁶

Maternal and fetal complications will not occur in all pregnant women with Degos disease, but frequent health check-ups are essential to monitor how the disease behaves during the pregnancy. 

Therapeutic dilemmas 

Currently, there are no established guidelines on how to manage Degos disease, due to the limited clinical research and the lack of large-scale studies. Therefore, preventive measures and treatments are not sufficiently studied in pregnancy, and their safety for the mother and baby remains unknown. 

Most healthcare professionals closely monitor pregnancies in women with Degos patients and advise management stratergies that balance the health of the mother and the safety of the baby. 

Real-world case insights

To date, there have been three reported pregnancy cases in Degos disease.

The first was reported in 1984. The patient had a successful pregnancy, with no complications for the mother or the baby. For the following 9 years, the daughter was medically followed and had only developed cutaneous lesions.⁷

The second Degos case was reported in 1997. This pregnancy led to the birth of a healthy baby. However, during the gestation period, the patient developed lesions, suggesting that pregnancy may have caused a disease flare-up.³

The third case was recorded in 2010. The patient had a smooth pregnancy and delivered a full-term baby without complications. She was examined 6 weeks postpartum and remained in good health, with no signs of symptom flare-up.³

Due to the rarity of Degos disease, reports of pregnancy cases in the current literature are limited. What we know so far is that with close medical monitoring and careful management, a pregnancy has the potential to proceed successfully. However, that is relevant to each individual case. In severe systemic Degos cases, poor pregnancy outcomes can occur. 

Living with degos disease during pregnancy

If you have Degos disease and are considering getting pregnant, there are a few things to keep in mind to minimise potential challenges as much as possible and ensure a safe pregnancy. 

• Early planning of the pregnancy with medical advice is key 

Degos disease can complicate a pregnancy; however, with careful planning and the support of a medical team, you can help ensure the best possible conditions for safely welcoming your baby.

• Importance of prenatal check-ups and possible hospital monitoring

During your pregnancy, various healthcare professionals may need to assess you and your baby, including a maternal-fetal medical specialist, a haematologist, and a dermatologist. In some cases, hospital monitoring may be recommended so these experts can closely assess you and your baby, share their insights, and work together on the best plan for your health and pregnancy.

• Importance of emotional and mental health support

It is normal for pregnant women to experience a wide range of emotions. Coping with Degos disease during this new chapter of life can cause additional concerns related to  the health and success of the pregnancy Support from family, friends and professionals is important. Mental health support, such as counselling or therapy, can also be a valuable resource during this time.

Summary

Degos disease is an extremely rare condition with only about 200 cases recorded worldwide since the disease was first described. Cases of pregnant women with Degos disease are even more rare, with only 3 cases reported in the current literature. Degos disease can make a pregnancy challenging for both mother and baby, and in extreme cases, may cause organ failure and subsequent miscarriage can occur. This may happen due to the nature of the disease, which involves blockage of blood vessels that deprives body parts of oxygen and, in the case of the placenta, nutrients too.

While a pregnancy with Degos disease can be challenging, with the right care plan and medical monitoring, positive outcomes are possible and evidenced. Planning is key to a successful pregnancy, and it’s essential to consult specialists before, during, and after pregnancy to help ensure the best possible health outcomes for both you and your baby.