What is Lofgren Syndrome?
Lofgren syndrome is a specific type of sarcoidosis, a health condition affecting several parts of the body that was first described in 1946 by Swedish lung specialist Sven Lofgren. This complex condition’s cause is unknown, but it commonly impacts the lungs and, regularly, the skin. Indeed, skin symptoms are found in up to 33% of people with sarcoidosis and can sometimes be the first sign of this disease.
Unlike standard sarcoidosis, which often develops slowly and becomes a long-term condition, Lofgren’s syndrome appears suddenly. It usually affects younger people and involves symptoms such as sudden outbreaks of red, swollen lumps on the skin (known as erythema nodosum), swollen lymph nodes in the area near the lungs, fever, and shifting joint pain, but without any involvement of granulomatous skin.
Lofgren’s syndrome usually indicates a good prognosis, meaning that people with this condition often have a positive health outlook.
What Causes Lofgren Syndrome?
The causes and the way skin sarcoidosis develops are not entirely clear, but both genetics and environmental factors play a role. Although skin sarcoidosis isn’t contagious, some people believe that certain cases can result from an unusual reaction to being exposed to infectious agents such as Mycobacterium tuberculosis or Propionibacterium, or environmental elements like beryllium, dust, or other factors related to certain jobs.
Risk Factors and Frequency for Lofgren Syndrome
Sarcoidosis, an illness that affects people of all ages and races, varies in occurrence and symptoms depending on a person’s gender, ethnicity, where they live, and their genetic makeup. In the United States, sarcoidosis affects more women than men, and is particularly common among African Americans. Around the world, the highest rates of sarcoidosis are found among Scandinavians, especially in Sweden and the United Kingdom, while the rates are lowest in Spain and Japan.
- Sarcoidosis impacts people from all demographics.
- In the United States, women and African Americans are most affected.
- The highest occurrence of sarcoidosis is in Scandinavia, particularly Sweden and the United Kingdom.
- The lowest rates of sarcoidosis are in Spain and Japan.
Lofgren syndrome tends to be more common in females, and is often found in Europeans of Scandinavian descent. It primarily affects adults between young and middle age, with the most common age being 37. Symptoms of Lofgren syndrome can differ by gender; for example, erythema nodosum (a type of skin inflammation) is usually found in women, while joint inflammation or arthritis is more common in men. This condition typically appears in the spring.
- Lofgren syndrome is more common in females and Europeans with Scandinavian descent.
- It mostly affects young to middle-aged adults, with an average age of 37.
- Erythema nodosum is more prevalent in women, while men often experience joint inflammation or arthritis.
- Lofgren syndrome usually shows up during the spring months.
Signs and Symptoms of Lofgren Syndrome
Lofgren syndrome is a medical condition that comes with a number of specific signs. These include: a skin condition called erythema nodosum, swelling of lymph nodes in the chest (bilateral hilar lymphadenopathy), fever, and a type of arthritis that moves around the body (migratory polyarthritis). Sometimes, it can also present with inflammation of the eyes (uveitis).
Interestingly, the arthritis associated with this condition is more common in men and affects the surrounding area of a joint with inflammation involving soft tissues and tendons. It usually affects both ankles at the same time, but it can also impact knees, wrists, and elbows.
The erythema nodosum part of Lofgren syndrome causes painful, red lumps under the skin. They are usually symmetrical and appear on the front of the lower legs. This is different from a skin condition called macular/papular sarcoidosis, which tends to show up on areas of the body that have been injured repeatedly. The skin lumps and fever generally improve on their own within six weeks, and they may heal with a change in skin color but without scarring or wasting away. The swelling in the lymph nodes may take up to a year to go away completely, but it does resolve entirely in 90% of cases.
Testing for Lofgren Syndrome
In diagnosing sarcoidosis, a condition that creates clumps of cells in your body’s organs, things can get a bit complex. This is because currently, there is no definitive test to confirm sarcoidosis. Doctors usually rely on understanding the patient’s medical condition, using imaging techniques like chest X-rays, and ruling out other diseases that cause similar symptoms. They also try to find the presence of non-caseating granulomas, which are clumps of cells that are a hallmark of sarcoidosis.
Sometimes, the patient’s observed symptoms alone can lead to a diagnosis without the need for a biopsy (a test where a small amount of tissue is removed for examination). One example of such a situation could be the combination of swollen lymph nodes in your chest, joint pain that moves from one joint to another, red or swollen skin (erythema nodosum), and fevers, a condition called Lofgren syndrome.
Validating a sarcoidosis diagnosis usually involves a chest X-ray, which primarily detects any lymph node swelling near your lungs. The X-ray might show a variety of conditions other than the textbook signs of sarcoidosis, like lymph node swelling on one side of your chest or even near your windpipe, with or without lung involvement.
Sometimes, if the disease isn’t following a typical pattern or doesn’t get better as expected, doctors may need to confirm the diagnosis using a tissue sample (biopsy). The most appropriate site for a biopsy needs to be determined for this. A thorough physical examination usually helps in this, with areas like your mouth, eyes, limbs, previous injury sites or tattooed areas, and lymph nodes carefully assessed. If a biopsy can’t be done on the surface of the body an invasive procedure might be required or even minimally invasive techniques like bronchoscopy (inspection of the inside of the lung’s airways).
In the diagnosis of sarcoidosis, certain cells’ ratios in the body have been useful as well. A higher ratio of CD4/CD8 cells, which are types of white blood cells, and an increase in IgG-secreting plasma cells (a type of immune cell) are often seen in sarcoidosis.
However, standard lab tests aren’t usually necessary in the diagnosis of sarcoidosis. The results can be rather general, with the possibility of finding a lower number of white blood cells, elevated calcium, specific protein levels, or other measures. It’s also crucial to rule out other diseases like tuberculosis that can mimic sarcoidosis symptoms. Finally, some reports hint at a connection between thyroid disorders and skin conditions in sarcoidosis, so checking thyroid function may be considered after diagnosing sarcoidosis.
Treatment Options for Lofgren Syndrome
Lofgren syndrome is usually managed with care aimed at helping manage symptoms, and it typically resolves naturally over a period of 1 to 2 years. Symptoms such as fever, fatigue, or joint pain can be treated with medications like non-steroidal anti-inflammatory drugs (NSAIDs), which are medications that reduce pain and inflammation, or colchicine, a medication commonly used to treat gout. In rare cases where the symptoms are severe, steroids may be needed. Steroids are strong anti-inflammatory drugs that can be gradually reduced over a few weeks to months.
What else can Lofgren Syndrome be?
If you’re experiencing symptoms like fever, joint pain, and skin lesions known as erythema nodosum, it might be due to sarcoidosis. However, these could also be due to a number of other factors:
- Certain medications like penicillin, sulfa drugs, oral contraceptives, and vaccines
- Various infections such as Streptococcus, different types of intestinal bacteria, Tuberculosis, or certain fungal infections
- Other diseases that cause inflammation in the body, like inflammatory bowel disease, lupus, Behcet’s disease
- Pregnancy
About 10% to 22% of erythema nodosum cases are related to sarcoidosis. To figure out the cause, doctors will study your medical history and may run tests to rule out diseases like tuberculosis. In cases where Lofgren’s syndrome (a set of symptoms including fever, joint pains and erythema nodosum) is suspected, specific x-ray findings can support the diagnosis. However, if the condition doesn’t follow the usual pattern, exploring other possible explanations and confirming the diagnosis with a tissue test may be necessary.
What to expect with Lofgren Syndrome
Lofgren syndrome has an excellent outlook and is associated with a more than 90% chance of naturally getting better within 2 years.