What is Lupus Miliaris Disseminatus Faciei?
Lupus Miliaris Disseminatus Faciei (LMDF) is a condition that usually affects the skin on the face, especially around the eyes. This medical term might sound complex, but this condition basically results in small, hard lumps under the skin. It shares some similarities with two other skin conditions – rosacea and sarcoidosis, but there are a couple of features that set it apart. Unlike sarcoidosis, it includes the formation of what doctors call ‘caseous necrosis’, which is basically a type of tissue death. And contrary to rosacea, people with LMDF don’t experience worsening of symptoms due to light exposure and there isn’t any redness involved.
Most of the time, LMDF clears up on its own over the course of a few years, but it can sometimes leave behind scars that change the way a person looks. It was first recognized as a specific condition back in 1878 by a doctor named Fox and his team.
The name of this condition actually has a historical link to tuberculosis because under the microscope, the skin changes look similar. However, some experts today suggest that a more accurate name would be ‘facial idiopathic granulomas with regressive evolution’. This hasn’t caught on widely yet, though. This condition has also been known under other names in the past, including micropapular tuberculid, Lewandowsky eruption, and lupoid rosacea. There’s another similar condition that impacts the armpit area called acne agminata, but most doctors consider LMDF to be its own distinct condition.
What Causes Lupus Miliaris Disseminatus Faciei?
The exact cause of LMDF, a skin condition, is uncertain and there are only about 200 reported cases. In the past, some believed this condition was related to tuberculosis because the symptoms were similar. Tuberculin skin testing, which checks for tuberculosis, is often negative for these patients. LMDF wasn’t found to be connected to tuberculosis in the lungs and generally doesn’t respond to the typical tuberculosis treatment, even though it has been tried in some cases.
Various studies and microscopic techniques have been unable to find M. tuberculosis, the bug that typically causes tuberculosis, within the skin changes seen in LMDF. There’s a chance that LMDF is linked to unknown bacteria not related to tuberculosis, though this hasn’t been confirmed. Other microscopic organisms, like those that contribute to acne and rosacea, have been considered as possible causes, but the connections are still being explored.
Similar to other skin conditions causing lumps on the face, it’s thought that damage to the hair follicle, possibly related to the immune system, leads to the hair follicle breaking down and causing the body to respond to skin parts like keratin, oils, or bacteria. This idea is supported by findings that show the immune response often focuses around a hair follicle. Whether the disruption begins with changes to the hair follicle or oil gland that result in a particular immune response isn’t known. The role of hormones affecting the hair follicle and oil gland, similar to some types of acne, hasn’t been established.
Risk Factors and Frequency for Lupus Miliaris Disseminatus Faciei
LMDF, or Lipedematous Miescher’s disease of the face, is a rare condition. So far, only about 200 cases have been documented. It’s seen among a wide range of ages but is most commonly found in young adults, with cases in children and older adults being less common. On average, the condition happens at around 33 years of age.
- LMDF affects people across a wide variety of ages.
- It’s most common in young adults, with an average age of 33.
- However, it can occur in both children and older adults, albeit less frequently.
- There’s no clear gender bias associated with LMDF.
- In some studies, women diagnosed with LMDF were older (average age: 43) than men (average age: 23).
- All patients over the mid-30s were women.
- Unlike rosacea, which shows gender predominance, LMDF does not show any gender specificity.
Signs and Symptoms of Lupus Miliaris Disseminatus Faciei
Patients commonly experience an abrupt appearance of small, red, yellow, brown, or skin-colored bumps on the skin. These bumps may or may not have pus and are often found symmetrically across the face. The bumps are predominantly in the middle of the face and can appear either on hair follicles or other areas of the skin. The lower eyelids are most often affected and it’s also common to see these on the forehead, cheeks, nose, upper lips, ears, chin, or neck. While it’s rare, these bumps can sometimes show up on other parts of the body like the trunk, limbs, and genitals. Generally, there are no other diseases associated with these symptoms.
Testing for Lupus Miliaris Disseminatus Faciei
If you have small, evenly spread, inflamed bumps or bumps filled with pus (papules or papulopustules) along with confirmed inflammation in tissue samples taken from these areas, your doctor may begin to suspect a condition called granulomatous rosacea. Other signs that might point towards this diagnosis include a reddish skin tone (erythema), visible blood vessels (telangiectasias), or a tendency to blush or flush easily.
Exposure to certain triggers such as use of steroids, sunlight, alcohol, or spicy foods might make these symptoms worse. To make sure this condition isn’t caused by an infection, your doctor might use special dyes (stains) or culture the tissue to look for bacteria or other infectious organisms, although these tests aren’t always perfect.
To help rule out another condition called sarcoidosis, many doctors will also check your blood levels of calcium and an enzyme called angiotensin-converting enzyme. They might order a chest x-ray and an eye exam as well. They may also perform blood tests to make sure you don’t have tuberculosis or certain types of infection.
Treatment Options for Lupus Miliaris Disseminatus Faciei
Choosing to monitor the disease without any medical treatment may lead to the condition resolving on its own within a year or two. However, there’s a good chance that significant scarring could occur during this period. There’s limited evidence on how to best treat this disease, but it’s generally agreed that some form of treatment should be considered even though the disease often gets better on its own.
This disease doesn’t usually respond well to the treatments typically used for a similar condition called granulomatous rosacea. For example, a type of antibiotic called tetracycline doesn’t seem to work well, while the response to a medication called isotretinoin has been mixed.
In one series of cases, patients responded well to oral prednisolone, a type of steroid medicine, or dapsone, another type of medication. Researchers also found that using these two medications together was particularly effective for patients who didn’t respond to either treatment alone. In the same series, combining dapsone with a medication applied to the skin called tacrolimus led to excellent results in all patients. Other research suggests that systemic corticosteroids, or steroid medications taken by mouth or injected, can also lead to good results.
There might also be other treatment options, such as clofazimine, medications typically used to treat tuberculosis, or a carbon dioxide laser treatment combined with a chemical peel. Tacrolimus could also be used with specific types of nonablative lasers. Starting effective treatment early may help reduce the risk of significant scarring.
What else can Lupus Miliaris Disseminatus Faciei be?
When examining skin conditions, doctors must consider a range of possible causes. These could include various types of infectious diseases like tuberculosis or infections caused by fungi, mycobacteria, treponema, or leishmania. However, if signs point away from an infection, doctors might consider conditions such as granulomatous rosacea or cutaneous sarcoid in their diagnosis.
Granulomatous rosacea and LMDF, a kind of skin disorder, can present similar symptoms like facial papules. However, there are significant differences too. For instance, LMDF can result in larger granulomas prone to a form of cell death called caseation necrosis, and doesn’t exhibit the characteristic skin damage, blood vessel dilation, or mite presence associated with rosacea. Another key difference is that LMDF, unlike rosacea, doesn’t come with phymatous changes (skin thickening), eye problems, or vascular symptoms like erythema (redness), flushing, or telangiectasia (small, widened blood vessels on the skin).
Typically, LMDF affects younger adults and can appear in teenagers and rare cases in children, with a slightly higher probability in men. While it is more likely than rosacea to disappear spontaneously, it can lead to significant scarring. Treatment responses of LMDF also differ from rosacea. It usually doesn’t respond well to antibiotics but reacts better to corticosteroids.
Sarcoidosis, a disease involving abnormal collections of inflammatory cells, should also be considered in the analysis. If a patient’s condition is slow to improve or shows progression, it is particularly important to rule out this condition. Even though LMDF has been suggested as a possible mild form of sarcoidosis, it doesn’t show the clinical, laboratory, or imaging evidence needed for a sarcoidosis diagnosis.
Other conditions to consider could be syringomas (sweat gland tumors), granulomatous perioral dermatitis, facial Afro-Caribbean childhood eruption, and other deep fungal infections. However, the presence of caseous necrosis in LMDF may steer the diagnosis away from these conditions.
What to expect with Lupus Miliaris Disseminatus Faciei
LMDF, which is a disease that only affects the skin, typically improves on its own over a period of several months to years. According to one study, the average duration of the disease is about 18 months.
Possible Complications When Diagnosed with Lupus Miliaris Disseminatus Faciei
The main long-term issue that individuals might face is significant scarring on the face, which may alter their appearance. Starting treatment early can help reduce or even prevent serious scarring.
- Significant facial scarring
- Potential disfigurement
- Possibility of reduction or prevention of scars with early treatment
Preventing Lupus Miliaris Disseminatus Faciei
Unlike rosacea, which is often linked to certain lifestyle habits, there is no evidence that certain lifestyle choices increase the risk of developing Linear Morphea in Frontoparietal Disorder (LMDF). After going through the necessary tests and procedures to confirm the diagnosis, patients should be comforted with the knowledge that this condition primarily affects the skin, and will eventually resolve on its own. Furthermore, it should be emphasized that treatment can lower the risk of permanent scarring.
