Belinda Z. Burgos González
Latin News Agency for Medicine and Public Health
The reason that led a 41-year-old Puerto Rican patient to the emergency room of the Mutual Aid Hospital was due to the fatigue she suffered. However, specialists were surprised by this unusual case and new diagnosis.
In an interview with Medicine and Public Health, Dra. Sherli Marie Martínez Rodríguez, an internist, narrated how rheumatological disease is known as atypical dermatomyositis in a Puerto Rican patient.
In Covid-19’s time, the strange condition led specialists to evaluate the patient who presented to the emergency room with fatigue, pain, inflammation and marks on his skin in the area of the hands, as the first what the medical team thought was that it was pneumonia due to the new virus and some infiltrations in the lungs that were found.
Dermatomyositis is a rare autoimmune disease that deals with an inflammation of the muscles that causes damage to muscle fibers and skin. It can cause red rashes on the knuckles, around the eyes or other parts of the body and can be associated with other diseases such as lupus and rheumatoid arthritis.
“The patient arrived with generalized edema, which had started gradually. However, she arrived because she had begun to have respiratory problems, or dyspnea. And then she tells us that since April, she had rashes on her body, she had problems with her joints and the edema led her to visit different doctors who had not been able to find what she had, ”he explained.
“Since we are in the covid pandemic, we thought it was that I had covid on top of whatever was happening. I did the test and it was not covid, it came out negative “he added.
After other tests, they noticed that the patient had Gottron papules, which are indurations in the rockets of the hands. This manifestation is a feature of dermatomyositis. The patient had edema in her hands, between her nails, the cuticle around her nails was inflamed and caused her a lot of pain.
“As these characteristics are typical of dermatomyositis, we try to look for the other characteristic that is classified in dermatomyositis, which is that the person has weakness in the proximal muscles.” narrated the doctor. However, the patient did not reflect this condition as a muscle weakness. So, the specialist focuses on whether the patient reflected symptoms of a subclassification of the disease called amyotrophic dermatomyositis.
“Amyotrophic dermatomyositis is when they have the characteristics of skin and pain but without muscle weakness. This disease is 10 to 30% of cases of dermatomyositis. The strange thing is that the person does not have muscle weakness, ”said Dr. Martínez Rodríguez.
In Puerto Rico, there is no specific date to talk about the number of people who have suffered from this disease. However, in the United States, 1 to 6 people can apply for every one hundred thousand adults. The special thing about this case was that it also represents a subclass of the disease, the patient presented two characteristics that are very rare in both scenarios.
“She had thrombocytopenia due to low platelets, a condition in the liver, and the disease of intensity in the lung. This is how dangerous it is because it can die quickly, ”said Dra. Martinez.
There are data that reflect previous atypical cases. However, due to the way in which this disease presents itself in the patient, Dra. Martinez indicated that because of the lung injury, doctors also doubted whether the patient had pneumonia or whether the lung was surrounded by the same dermatomyositis.
After the sputum test to determine the presence of bacteria in the patient and various laboratory tests that were negative, having no data to confirm that the patient suffered from pneumonia, the doctors concluded that the involvement in the lungs was it was due to a dermatomyositis attacking the lung.
This disease manifests itself in the patient, and affects other organs of his body, specifically the liver with high liver enzymes.
In short and the most amazing thing for specialists is that the muscular condition of this rheumatic disease began causing damage to other organs of the patient’s body.
“We will start treating her and her lungs will start to heal. The disease returned when he started being treated with high-level steroids, he was bombarded with steroids, he was given antinuclear drugs that attacked the immune system to lower him a bit. And as soon as we did that the lung began to recover. Then, we had another basis that yes, it was dermatomyositis attacking the lung “, argued Dr. Martinez.
When the patient sold this painting, he received the assistance of a multidisciplinary team composed of 7 doctors: a rheumatologist, an infectologist, a pulmonologist, an internist, a hematologist oncologist, a gastroenterologist and a doctor of physical medicine and rehabilitation .
The clinical lesson for specialists is based on the importance of having seen such an atypical case. Especially because it has already been noticed that it is possible for dermatomyositis to attack the lung, and the lethality is high and standing at 60%. “So it is important to be able to quickly identify the type of dermatomyositis for this reason, because as it is not something so common we understand that it can go unnoticed and the consequence of this could be the death of a patient. And also knowing this would allow us to raise data more fast “emphasized Dr. Martinez.
The patient managed to recover after remaining hospitalized for a month and a half. Even, due to the complications of the specialists, he came to think that they would have to tube the patient. But, it was not necessary, and the patient survived in a complex, rare, and unusual scenario.
“Something important is that these cases are also related to malignancy. She had no sign that she had any cancer. The doctor insisted.
Dra. Martinez said the problem is that rheumatic diseases are usually inflammatory, leading the patient to express when a joint hurts but if the pain goes away, and people take a long time to determine what the causes are, the diagnosis may vary. The delay can be further complicated by the person suffering from any disease associated with this field of health, especially if the patient suffers from nonspecific patterns.
Therefore, the internist insisted that it is important that people attend their routine cities with the general practitioner, because they are the ones who know the patient best, can detect these manifestations and can refer the patient to the relevant specialist if necessary.
“This is often more prevention than the consequences. Medicine works when we prevent it and the general practitioner, our family doctor is the one who has the tools to suspect something and take us to the relevant specialist. ”Concluded Dr. Martinez.