What is Jessner Lymphocytic Infiltration of the Skin ?
Jessner lymphocytic infiltration of the skin (JLIS) refers to a rare, harmless skin condition. It typically shows as raised bumps or patches (papular or plaque-like eruptions) primarily on sun-exposed areas like the face, neck, and trunk. These patches are reddish (erythematous), do not have any scales, and can also form circular shapes (annular).
This condition takes a slow course, lasting weeks to months. As the patches spread outwards, they sometimes heal or clear up in the center. Although these outbreaks can disappear on their own or with the help of medications, they often come back. While the lesions are generally painless, some people may experience sensations of burning and itching (pruritus).
The diagnosis is confirmed by using a biopsy, a procedure where a small piece of skin is removed for examination under a microscope. This reveals specific types of cells (known as plasmacytoid monocytes or plasmacytoid dendritic cells) around the blood vessels and sweat-producing glands of the dermis, the second layer of the skin. Occasionally, these cells could extend into the subcutaneous tissue, the layer beneath the dermis. These cells are often involved in autoimmune diseases and other skin disorders. Their presence, along with an unusually large number of a type of white blood cells called CD8+ T cells, confirms a JLIS diagnosis. However, we still don’t know what causes this disorder, but there may be genetic and autoimmune factors involved.
JLIS, also recognized as benign lymphocytic infiltration of the skin, Jessner-Kanof syndrome, and benign chronic T-cell infiltrative disease, was first described in 1953. By 1969, it was grouped under a broader term ‘cutaneous lymphoid hyperplasia’, which also included different skin diseases with similar cell patterns. There’s ongoing debate about whether JLIS is a unique disease or just a part of a range of skin diseases, like discoid lupus erythematosus, polymorphous light eruption, and possibly malignant lymphoma. However, no solid evidence supports that it progresses into any of these diseases, although some cases show concurrent symptoms.
What Causes Jessner Lymphocytic Infiltration of the Skin ?
The cause of Jessner lymphocytic infiltration of the skin (JLIS), a specific skin condition, is still not fully understood. However, researchers speculate that a variety of factors may play a role. Some people with JLIS have family members with the same condition, suggesting a possible genetic or hereditary link.
Another theory suggests that JLIS might be linked to autoimmune diseases, where the body’s immune system mistakenly attacks healthy cells. For instance, specific immune cells known as plasmacytoid dendritic cells, which are active in JLIS, are also involved in an autoimmune disease known as systemic lupus erythematosus. Therefore, some experts wonder if JLIS might be an early sign of this disease or a progression of a mild form of lupus erythematosus.
There’s also some evidence that the immune system’s natural killer cells, which help to defend against diseases, might not work as effectively in people with JLIS. This is something often seen in cancer and disorders affecting the body’s hormone system.
Besides, some studies suggest that sun exposure, or over sensitivity to sunlight, might trigger JLIS. This theory is further supported by the fact that symptoms generally appear on areas of the skin exposed to the sun. In some cases, specific medications or treatments, such as angiotensin-converting enzyme inhibitors (a type of blood pressure medication) and certain drugs used to suppress the immune system, may induce JLIS. Other less common triggers mentioned in medical reports include a specific type of medication called duloxetine, HIV and a type of infection caused by the bacterium Borrelia burgdorferi.
Risk Factors and Frequency for Jessner Lymphocytic Infiltration of the Skin
Jessner lymphocytic infiltration of the skin (JLIS) is a rare skin condition and we don’t know how many people it affects in both the U.S. and around the world. It typically starts between 30 and 50 years of age, but it can also occur in children, though that is less frequent. Initially, it was thought to affect more males, but recent studies show that it impacts males and females equally. There’s also no evidence that it affects one race more than others.
Signs and Symptoms of Jessner Lymphocytic Infiltration of the Skin
Jessner lymphocytic infiltration of the skin (JLIS) is a condition where patients usually have painless, red skin patches or bumps. These often appear on exposed areas like the face, neck, and body. These patches or bumps may have been present for weeks to months. Sometimes, these skin changes begin or worsen after being out in the sun. The skin changes may disappear on their own without causing any problems and then come back in the same place or in a new place. Although these skin changes usually don’t hurt, some patients might feel a burning sensation or itchiness. It’s important to ask about similar skin conditions in the family, as some cases suggest it can be inherited. Also, as JLIS may be connected with an autoimmune response, patients should be asked about their own and their family’s history of autoimmune diseases.
When a doctor examines the skin changes, they’ll find them to be clearly defined. They might appear ring-shaped or seem to be growing outwards. The skin changes are not flaky and there’s no sign of hair follicle blockage or thinning. The skin changes can be shaped in crescents or rings, ranging from 2 mm to 2 cm across. A thorough inspection of the skin should be done since there could be multiple skin changes. They most often appear on the cheekbone area of the face and the upper back. However, they can show up on any part of the body, including the forehead, neck, area behind the ear, arms, legs, and body.
Testing for Jessner Lymphocytic Infiltration of the Skin
When evaluating a patient for an autoimmune disorder, the doctor would typically begin with several basic laboratory tests such as complete blood count (which measures different components of blood, including white and red blood cells) and urinalysis (a test that checks for different substances in urine).
In addition, a special panel of tests known as an autoimmune workup is also needed. These tests detect the presence of certain antibodies in the patient’s body, which are proteins produced by the immune system to fight off harmful substances. The first test to be done are the Antinuclear antibodies (ANA) and erythrocyte sedimentation rate (ESR), which help identify inflammation in the body.
If these tests come out positive, the doctor will recommend further testing for conditions such as systemic lupus erythematosus, antiphospholipid syndrome, Sjogren’s syndrome, and rheumatoid arthritis.
Another test that might be needed is the UVA and UVB provocative photo testing. This is a type of test that exposes your skin to ultraviolet light to see how it reacts, which can help in diagnosing certain skin conditions.
Finally, diagnostic testing may include a skin biopsy. This involves taking a small sample of skin tissue, which is then examined under a microscope using different types of staining techniques. These stains help to highlight certain components of the cells, making it easier to identify any abnormalities.
Treatment Options for Jessner Lymphocytic Infiltration of the Skin
Currently, there’s no definitive treatment for Jessner lymphocytic infiltration of the skin (JLIS), a benign skin condition. Common treatment options include creams containing steroids like hydrocortisone, triamcinolone, betamethasone, and prednisone or drugs for malaria like hydroxychloroquine. However, because these medications can have harmful side effects, many people choose not to take them for a non-dangerous condition.
Steroid creams and injections are usually the first choice of treatment. If the condition makes the skin sensitive to sunlight, antimalarial medications can be more effective. Other drugs, like tacrolimus, thalidomide, etretinate, methotrexate, oral auranofin, and proquazone have been reported to be helpful in some cases.
Other treatments focus on managing the condition’s appearance rather than curing it. These include cosmetic camouflage to conceal the affected areas, protecting the skin from sunlight, and removing small lesions through surgical procedures. Some other less commonly performed treatments include laser therapy, photodynamic therapy which uses light to treat the skin, and cryotherapy, which uses cold temperature to destroy abnormal tissue.
What else can Jessner Lymphocytic Infiltration of the Skin be?
Jessner lymphocytic infiltration of the skin, or JLIS, is part of a group of conditions that involve the presence of a particular type of immune cells, called lymphocytes, in the skin layer known as the dermis. There are four other conditions that have similar features as JLIS. These five conditions are often grouped together and are known as the ‘5 L’s’. Here’s a quick rundown of the four other conditions:
- Polymorphous Light Eruption (PLE): JLIS can be distinguished from PLE by the appearance of active skin lesions during winter or in areas that don’t get sunlight.
- Discoid Lupus Erythematosus/Lupus Erythematosus Tumidus: Tests for certain substances in the blood, such as ANA, anti-smith, and double-stranded DNA, can help differentiate JLIS from lupus erythematosus. JLIS also typically has less involvement of the torso, a higher proportion of a type of lymphocytes known as B cells, and is less common in females due to lupus’s autoimmune nature.
- Well-Differentiated Lymphocytic Lymphoma: Unlike JLIS, lymphoma can affect the bone marrow while JLIS is limited to the skin.
- Lymphocytoma Cutis: Lymphocytoma cutis usually doesn’t have detectable B cells, unlike JLIS.
Besides the ‘5 L’s’, other conditions that need to be considered when diagnosing JLIS include Cutaneous lymphoid hyperplasia, Granuloma annulare, Granuloma faciale, Gyrate erythema, and Reticular erythematous mucinosis. The latter condition can be identified with a special stain for dermal mucin, a substance not found in JLIS.
What to expect with Jessner Lymphocytic Infiltration of the Skin
Jessner Lymphocytic Infiltration of the Skin (JLIS) is a harmless condition with a great prognosis, meaning it’s expected to have a positive outcome. The condition can actually go away on its own without leaving any lasting effects. Importantly, having JLIS does not increase your risk of dying.
Possible Complications When Diagnosed with Jessner Lymphocytic Infiltration of the Skin
Jessner’s disease, officially known as Jessner lymphocytic infiltration of the skin (JLIS), can impact the skin around the eyes. This can cause a severe condition called cicatricial ectropion, where the lower eyelid turns outward.
Common Effects:
- Impact on skin around the eyes
- Severe cicatricial ectropion (outward turning of the lower eyelid)
Preventing Jessner Lymphocytic Infiltration of the Skin
Jessner lymphocytic infiltration of the skin (JLIS) is a condition with unknown causes, so there’s no definite way to prevent it. However, experts believe that exposure to sunlight may cause this skin condition, so patients are usually advised to limit their sun exposure and protect their skin against the sun.
Patients who suffer from periorbital lesions (abnormal tissues around the eyes) often face a common complication called ectropion, where the lower eyelid turns outwards. If you have this condition, your doctor will want to keep a close watch on you to manage this complication effectively.
Lastly, if you’re taking certain treatments like topical steroids (cream or ointment that contains medicine to reduce inflammation) and antimalarials (medications that prevent or treat malaria), you’ll need to visit your doctor regularly. This is crucial, as they need to check and ensure you’re not experiencing harmful side effects – such as skin atrophy (thinning or weakening of the skin) – due to these medications.
