Lichen planopilaris: A closer look at "follicular hyperkeratosis"

Follicular hyperkeratosis = Perifollicular scale

Lichen planopilaris (also known as "LPP") is a scarring hair loss condition. Individuls affected by the condition develop hair loss, increased hair shedding along with scalp itching, burning and pain.

Scaling in LPP

An up close examination with dermoscopy is shown here and shows classic features including white scale around hairs in early stages. This scale is known as "perifollicular scale" or "follicular hyperkeratosis." This scale is often prominent in active stages of the disease. It can be reduced by treatment and even reduced to some extent by a good shampooing of the scalp.

Treatments for LPP

Treatments include topical steroids, steroid injections, topical calcineurin inhibitors, and oral treatments such as doxycycline, hydroxychloroquine (Plaquenil), methotrexate, mycophenolate mofetil, cyclosporine, and others. Lasers, including the 308 nm excimer laser may also provide benefit.

Is Lichen Planopilaris Caused By A Fungus?

Lichen planopilaris is the long name given to a type of scarring hair loss condition. It is sometimes referred to as follicular lichen planus. The name "lichen" comes from the skin lesions of lichen planus that some patients with lichen planopilaris also have. The skin lesions are flat just like lichens that one might see walking in the forest. 
Lichen planopilaris is not due to a fungus. It is an autoimmune inflammatory condition that causes permanent hair loss. Treatments include anti-inflammatory agents not antifungal agents.

Hair transplants for Scarring Alopecia

How successful are hair transplants in scarring alopecias?

This is an in important question and one that needs good data. I serve as the chair of a committee of the International Society of Hair Restoration Surgery. On Friday we sent out a survey to hair transplant surgeons around the world with the hopes of gathering more information on the successes and failures surgeons have had when transplanting scarring alopecias.

There is no doubt that hair transplantation works wonderfully in some patients with scarring alopecia and does not work well in others. One must always have quiet (inactive) disease for at least 1-2 years before a transplant is attempted.

 

What are the criteria for transplanting scarring alopecia?

In general, a scarring alopecia must be quiet for 1-2 years before a transplant can be even considered. Several years ago I put forth criteria for determining if an individual with lichen planopilaris is a hair transplant candidate:

 

Criteria for Hair Transplantation Candidacy in LPP

1.  The PATIENT should be off medications.

Ideally the patient should be off all topical, oral and injection medications to truly know that the disease is burnt out and ‘inactive’. However, in RARE cases, it may be possible to perform a transplant in someone using medications AND who meets criteria 2, 3 and 4 below.  This should only be done on a case by case basis and in rare circumstances as the risk for disease reactivation is high.  A patient using medications to suppress disease activity is at high risk for reactivation following hair transplant surgery. It is a last resort in a well-informed patient.

2. The PATIENT must not report symptoms related to the LPP in the past 12 months, (and ideally 24 months).

The patient must have no significant itching, burning or pain. One must always keep in mind that the absence of symptoms does not prove the disease is quiet but the presence of symptoms certainly raises suspicion the disease could be active.  Even the periodic development of itching or burning from time to time could indicate the disease has triggers that cause a flare and that the patient is not a candidate for surgery. The patient who dabs a bit of clobetasol now and then on the scalp to control a bit of itching may also have disease that is not completely quiet. 

3. The PHYSICIAN must make note of no clinical evidence of active LPP in the past 12 months, (and ideally 24 months).

There must be no scalp clinical evidence of active LPP such as perifollicular erythema, perifollicular scale (follicular hyperkeratosis). In addition, the pull test must be negative. 

4. Both the PATIENT and PHYSICIAN must show no evidence of ongoing hair loss over the past 12 months (and ideally 24 months). 

There must be no further hair loss over a period of 24 months of monitoring off the previous hair loss treatment medications. This general includes the patient and physician's perception that there has been no further loss as well as serial photographs every 6-12 months showing no changes.  As discussed above, the 12 month waiting time is the standard of care as an accepted definition for hair transplant candidacy.

5. The patient must have sufficient donor hair for the transplant.

Not all patients with LPP maintain sufficient donor hair even if the disease has become quiet. 

 

Disease Reactivation Following Surgery

My research has focused on the chances of reactivation of LPP after surgery. It is important to be aware that ANY patient with LPP is at risk for reactivation or a 'flare' of their LPP after surgery.  The risk, I estimate, is as follows:

LPP Reactivation Risk (Donovan, J, unpublished data)

i)               A patient with active LPP before their transplant is nearly guaranteed to have a flare of his or her LPP if a hair transplant is done. (estimate 90-100 % chance of flare within 2 years post transplant)

ii)             A patient with partially active LPP before their transplant is very likely to have a flare if a hair transplant is done. (estimate 70-90 % chance of flare within 2 years post transplant)

iii)            A patient with medication induced inactive LPP before their transplant has a moderate chance of a flare if a hair transplant is done (estimate 50-70 % chance of flare within 2 years post transplant)

iv)            A patient with inactive LPP off all medications for 1 year before their transplant has a low chance of a flare if a hair transplant is done (estimate 10-25 % chance of flare within 2 years post transplant)

v)             A patient with inactive LPP off all medications for 2 years before their transplant has a low but definite chance of a flare if a hair transplant is done (estimate less than 10% chance of flare within 2 years post transplant)

Atrophy in FFA: Is it from my topical steroids?

Atrophy in FFA is often from the disease itself 

A common concern from patients with FFA is that their steroids caused atrophy. By atrophy we mean thinning of the skin. Patients with atrophy have thin skin, visible veins. In FFA atrophy leads to blue veins becoming easy to see throughout the frontal scalp and especially at the temples. Patients want new options for treating the disease because they are worried about the atrophy.

FFA Causes Atrophy

There is one assumption that is often wrong here - and that is that steroids are the sole cause of atrophy in FFA. MOST of the time the steroids are not the main cause of the atrophy ! It is very important to keep in mind that the disease itself causes atrophy and visible veins. It is certainly very true that the steroids can cause atrophy too. But FFA itself is usually the leading cause of atrophy in patients with FFA. Many many patients with FFA who have never used steroids can have atrophy - some severe. In fact, severe atrophy is one of the so called poor prognosis signs in FFA. 

Treatment Considerations for Patients with Marked Atrophy

When patients show a considerable amount of atrophy, I usually try to limit this by using non steroids instead of steroid. Non steroids such as pimecrolimus (Elidel) and tacrolimus (Protopic) do not cause atrophy. They seem equivalent although no comparison studies have been done.  My previous research has also shown that finasteride and dutasteride may actually reverse atrophy in a proportion of patients. 

Frontal Fibrosing Alopecia (FFA)

Other than the Front, What is Affected?

Frontal fibrosing alopecia (FFA) is a scarring alopecia that affect women to a much greater extent than men. In FFA the frontal scalp is typically affected. However, the name does not capture the full extent of the hair loss. Patients with FFA frequently develop hair loss around the back of the scalp (behind the ears and very back above the neck), and frequently in the middle of the scalp as well. Eyebrows, eyelashes, arm hair, leg hair, underarm hair and pubic hair are frequently affected.

Partially Treated Lichen Planopilaris

 Scale Gone, Redness Remains

Lichen planopilaris (LPP) is a scarring hair loss condition. The goal of treating LPP is to stop the condition. Successful treatment is associated with a halting of hair loss but also with an improvement in the symptoms and signs of the disease. Patients will notice a reduction in itching and burning and clinically there will be an improvement in scaling and redness around hairs. Sometimes scaling is the first to improve and improvements in redness happen later. This picture shows a patient with partially treated lichen planopilaris. The disease is still active although scaling has improved. The patient's itching has also improved.

Sea of Singles (SOS): A Potential Sign of Scarring Alopecia

Lichen planopilaris (LPP) is a type of scarring hair loss condition. Patients frequently present with scalp itching, and sometimes scalp burning and tenderness. Increased hair shedding is common in the early stages. Hair loss is generally permanent and treatment helps stop the disease or at least slow down progression.

Clinically, dermoscopy (trichoscopy) of LPP often shows perifollicular erythema and perifollicular scale (follicular keratosis).

These findings are not present in all forms of LPP. A less common presentation of LPP is shown in the photo. Patients have hair loss with scalp itching. However, by dermoscopy they have many single hair follicles growing in a base of redness. This is what I have termed the "sea of singles" (SOS) appearance to describe the numerous single hairs and absence of hair follicle units containing 2 and 3 hairs. This form of LPP is similar to Abbasi's subtype described in 2016 and fibrosing aloepcia in a pattern distribution described by Zinkernagel in 2000. The "SOS" trichoscopic appearance is important to remember and provides a clue that the patient may have a scarring alopecia.

 

Reference

Zinkernagel MS et al. Arch Dermatol 2000

Abassi A et al. Dermatol Surg. 2016.

Oral Immunosuppressants for Lichen planopilaris: should I increase my dose?

Dosing oral immunosuppressants for Lichen planopilaris (LPP)

There are many different immunosuppressants and immune modulators that can be used for treating lichen planopilaris. Examples include doxycycline, hydroxychloroquine, methotrexate, mycophenolate, cyclosporine.  I'm often asked what dose a patient should be using? 

What dose should a patient be using? 

When it comes to immunosuppressant medications, I always try to keep patients on the lowest possible dose that controls their disease. Generally I start at fairly standard doses of immunosuppressants and observe what happens to the patient's hair loss. For example, this might be 200 or 400 mg of hydroxychloroquine (Plaquenil) daily, 15-20 mg of methotrexate weekly, 150-300 mg of cyclosporine, 500-1000 mg of mycophenolate mofetil, 100 mg of doxycycline. If the disease is vastly improved after a few months, we may consider going down on the dose or staying at the same dose for a few more months. If the disease is getting worse, we might consider going up on the dose is their is room to go up or changing the immunosuppressant altogether. 

Nausea with Doxycycline: What strategies can help reduce nausea?

Doxycycline and Nausea

Doxycycline is an antibiotic. It's used of course in treating infections but it is commonly used for a variety of scarring alopecias including lichen planopilaris, frontal fibrosing alopecia, central centrifugal cicatricial alopecia, folliculitis decalvans and sometimes dissecting cellulitis.

The drugs has two important properties: it stops infection and reduces inflammation. For some conditions such as lichen planopilaris, it's the anti-inflammatory properties that are useful. For other conditions such as folliculitis decalvans, it's the anti-bacterial and anti-inflammatory properties that are key. 

The drug has a number of potential side effects even though it is generally well tolerated for most. It can cause nausea, vomitting, sun sensitivity, headaches, increased chance of yeast infections in women, rash. 

Doxycycline and Nausea

Some patients developed considerable nausea with doxycycline. Some will even vomit.  This can be a short term issue for some users which improves over time. For others it is something that continues and may even require the patient to stop the medication.  Anyone with nausea from doxycycline should speak to their prescriber for advice on how to reduce the nausea. 

Tips to reduce nausea

1.  Take doxycycline with food. Unlike tetracycline, doxycycline still gets absorbed quite well into the blood stream if the patient takes it with food. The food intake really helps to reduce nausea and this should be encouraged

2.  Avoid spicy foods with the doxycycline. Anything that upsets the stomach has the potential to makes things worse with doxycycline. I generally recommend avoiding spicy foods with doxycycline. 

3. Take Gravol.  If nausea continues despite food intake, dimenhydrate (Gravol) can be used 1 hours before the doxycycline is taken. I generally recommend starting with 25 mg Gravol and then 50 mg and then 100 to see what dose can help reduce the nausea. Gravol can make people drowsy and sleepy so this needs to be considered if one is driving or doing anything that requires focus. 

4. Use Ginger. Ginger is also a helpful anti-nausea treatment. There are a number of candies, lozenges on the market that contain ginger and can be used prior to the patient taking the doxycycline. The company that makes Gravol also has a product "Ginger-Gravol" which can be very helpful. this does not contain Gravol and therefore does not cause drowsiness.

5. Reducing the doxycycline dose. For some users, the nausea is dose related. Reducing the dose can help.

Tetracyclines for Scarring Alopecia: Which one should I use?

Tetracycline Antibiotics for Scarring Alopecia

Tetracycline, Doxycyline and Minocycline are members of the tetracycline family of antibiotics. These drugs are commonly used to fight infection but are also frequently used for their anti-inflammatory effects and are therefore used in a variety of scarring alopecias including lichen planopilaris, frontal fibrosing alopecia, and pseudopelade. 

These medications have several well known mechanisms for halting inflammation: they inhibit matrix metalloproteinases, they inhibit angiogenesis, they have antioxidant effects and they block the production of various pro-inflammatory cytokines. 

In terms of treatments for lymphoctic scarring alopecias, all these drugs are fairly similar in terms of efficacy but good studies have yet to be published. Personally I prefer doxycycline over others. 

Doxycycline is the most commonly prescribed tetracycline family member. It can be taken with food and tends to have the least overall chances of side effects compared to minocycline and tetracycline. That is not to say of course it does not have side effects because it certainly does. Doxycycline can cause upset stomach, headaches and tends to be the most photosensitizing.  Headaches and raised intracranial pressure are a rare side effect but nevertheless must be respected. Immediate cessation of the drug and medical attention is needed in anyone with persistent headaches on doxycycline. Women using doxycycline are at increased risk for vaginal yeast infections. The dose is 100 mg once to twice daily. The medication should be taken while seated upright and with plenty of water to avoid heartburn and esophagitis. Doxycycline is safer than tetracycline (see below) for use in those with kidney disease.

Recently, the possible use of low dose sub-antimicrobial doses of doxycycline have emerged on the market. This is sometimes referred to as "SD" or sub-antimicrobial dosing." Such medications are frequently used for inflammatory conditions such as rosacea. I frequently use these drugs in patients with scarring alopecia who I want to transition off higher doses doxycycline. Doxycycline formulation Oracea was approved by the FDA in 2006. Side effects are less than 100 mg conventional doxycycline but may not be appropriate as a first line off label treatment for active scarring alopecias.

Tetracycline is less expensive than doxycycline which is great but it needs to be taken on an empty stomach.  This makes it less convenient. The dose is typically 500 mg twice daily for typical dosing in lichen planopilaris but this can sometimes be increased to three times daily. It must not be used in those with kidney disease and used only with extreme caution in those with liver disease. It must never be used during pregnancy and never in children (particularly under 8 years due to effects on teeth and bones). Tetracycline is less photosensitizing than doxycycline but caution is still needed. Tetracycline is more photosensitizing than minocycline.

Minocycline can sometimes be associated side effects that are not seen commonly with other tetracycline members including a serious lupus like phenomenon and other side effects like malaise and joint pains. Minocycline is a much more frequent cause of serious reactions like hypersensitivity reactions, serum sickness like reactions and single organ dysfunction. Pigmentation issues are also possible. It is less photosensitizing compared to doxycycline and tetracycline. The dose is 100 mg daily and doses up to twice daily may also be considered. Similar to tetracycline and doxycycline, minocycline must never be used in pregnancy.

All in all, one should speak with his or her dermatologist about other specific side effects of the tetracycline group. These medications may be taken for many months to even several years in those with scarring alopecia. Patients should not use retnoid medications while using tetracyclines. Moreover one should not consume iron, magnesium, calcium or aluminum at the same time as their tetracycline as these bind to the tetracycline and block absorption. Tetracyclines (all 3 members) must never be used during pregnancy and never by children under 8 due to teeth discoloration.

Dissecting Cellulitis (DSC)-Healed Sinus Tracts

Dissecting Cellulitis (DSC), is a rare scarring hair loss condition that is characterized by deep inflammation and leads to the formation of draining sinus tracts (especially tunnels that allow pus and inflammation to escape). The diagnosis of DSC in advanced stages is easy as these openings (sinus tracts) can be seen all over the scalp. In early stages, up close exam and use of a dermatoscope can prove extremely helpful.

Early DSC is characterized on dermoscopy by large yellow dots, thin vellus hairs within the area, broken hairs and healing (covered) or open sinus tracts. This picture shows a sinus tract at an earlier stage than the picutre yesterday (panel 4 in our 5 day series). There is inflammation in the skin which gives a red color.

Dissecting Celluliti(DSC)

Healed Sinus Tracts

We will continue our week's theme of Dissecting cellulitis (DSC), a rare scarring hair loss condition. It is characterized by deep inflammation and leads to the formation of draining sinus tracts (especially tunnels that allow pus and inflammation to escape). The diagnosis of DSC in advanced stages is easy as these openings (sinus tracts) can be seen all over the scalp. In early stages, up close exam and use of a dermatoscope can prove to be extremely helpful.

As seen yesterday, early DSC is characterized on dermoscopy by large yellow dots, thin vellus hairs within the area, broken hairs and healing (covered) or open sinus tracts. This picture shows a healed sinus tract (arrow).

Dissecting cellulitis (DSC)

Dissecting cellulitis (DSC) is a rare scarring hair loss condition. It is characterized by deep inflammation and leads to the formation of draining sinus tracts (especially tunnels that allow pus and inflammation to escape - see number 1 and 4 in the picture). The diagnosis of DSC in advanced stages is easy as these openings (sinus tracts) can be seen all over the scalp. In the early stages an up close exam and use of a dermatoscope can prove extremely helpful.

Early DSC is characterized on dermoscopy by large yellow dots, thin vellus hairs within the area, broken hairs and healing (covered) or open sinus tracts. The early stages of the nodule can mimic alopecia areata (see top right, number 3 and 5). A swiss cheese like appearance is common as scarring progresses (number 2). Biopsies of DSC often show deep inflammation but in more advanced cases show inflammation higher up in the skin which can easily be mistaken for another scarring alopecia known as "folliculitis decalvans." Therefore, it is not uncommon for patients to be referred with a diagnosis of biopsy "proven" folliculitis decalvans only to need to explain to them after examining their scalp that what they actually have is DSC.

Minoxidil in FFA: Does it help?

I frequently get asked whether minoxidil has any benefit in treating frontal fibrosing alopecia (FFA). It seems that it could provide some benefit but it's not completely clear yet if it is truly helping the patient's FFA or their underlying androgenetic alopecia that many patients with FFA also have. Large scale studies are needed. 

I generally add minoxidil once I have some evidence that a patient is stabilizing with their main anti-inflammatory treatment. This typically includes one or more of topical steroids, steroid injections, doxycycline, hydroxychloroquine and anti-androgens such as finasteride or dutasteride. 

It’s interesting that 32 % of patients in one study had an improvement in their FFA with use of anti-androgens. When one looks at a larger group of 111 FFA patients of which 74.8 % were using minoxidil, one notes that 47 % of patients had an improvement with anti-androgens. So it does seem that patients using minoxidil had better outcomes. There is at least some suggestion here that minoxidil might help. 

Conclusion

Up to 40 % of patients with FFA have androgenetic alopecia so it’s difficult sometimes to decipher whether minoxidil is truly helping the patient’s FFA or whether it is helping their underlying androgenetic alopecia. More good studies are needed.

Reference

Vano-Galvan S et al. Frontal fibrosing alopecia: a multicentre review of 355 patients. J Am Acad Dermatol 2014; 70: 670-678

Is sunscreen use more common in men with FFA?

This is a controversial topic but this study (as well as a study of FFA in women) has caught the attention of many. A study by Kidambi et al compared how 17 men with FFA and 73 men without FFA responded to a lengthy survey. FFA is relatively rare in men but information on a link to sunscreen use was important to investigate given the possible role among women.

A much greater proportion of men with FFA reported using sunscreens (as well as facial moisturizers) at least twice weekly compared to men without FFA. Specifically, 35 % of FFA patients reported such sunscreen use compared to just 4 % of men without FFA.
 

Conclusion

We have a long way to go to definitely prove sunscreens have a role. But two studies now (one in men and one in women) have described potentially the first environmental factor implicated in the way FFA develops. An environmental factor is certainly thought to be responsible given that FFA was relatively unheard of 20 years ago. There are more good studies that are needed.
 

Reference

Aldoori N et al. Frontal fibrosing alopecia: possible association with leave-on facial skin care products and sunscreens; a questionnaire study. Br J Dermatol 2016.

Kidambi AD et al. Frontal fibrosing alopecia in men: an association with facial moisturizers and sunscreen. Br J Dermatol 2017.

Is sunscreen use more common in women with FFA?

This is a controversial question, there has been one study that has caught the attention of physicians and patients around the world. A study by Aldoori et al compared how 105 women with FFA and 100 women without FFA responded to a lengthy survey.

Surprisingly, a much greater proportion of women with FFA reported using sunscreens (at least twice weekly) compared to women without FFA. Specifically, 48 % of FFA patients reported such sunscreen use compared to just 24 % of women without FFA.

Conclusion

We still have a long way to go to definitely prove sunscreens have a role. It is potentially the first environmental factor implicated in the way FFA develops. An environmental factor is certainly thought to be responsible given that FFA was relatively unheard of 20 years ago. More good studies are needed.

Reference

Aldoori N et al. Frontal fibrosing alopecia: possible association with leave-on facial skin care products and sunscreens; a questionnaire study. Br J Dermatol 2016.

Does smoking prevent FFA?

The short answer is that we do not know. A 2017 study by Fonda-Pascual suggested that smoking was somehow protective against the development of FFA and that non-smokers had more severe disease. Other studies, including a study by Dr Messenger's group from the UK did not show this link.

Conclusion

To date, there is no solid information available to suggest that smoking either causes FFA or prevents the development of FFA. Large scale studies will help answer the question for good.
 

References

Aldoori et al. Br J Dermatol 2017.
Fonda-Pascual et al. JEADV 2017.

If hair extensions are causing hair loss, do they need to be removed?

Hair extensions can sometimes cause hair loss. Whether to remove the extensions or change the type of extension is a decision made on a case by case basis. This is not always a simple answer. Sometimes the improvements that come with the patient using the extensions supercedes a small amount of hair loss that might come with wearing them. This makes removing the extensions less relevant - especially if this is a more permanent type of camouflaging option for the patient. If, however, the hair extensions are causing significant hair loss and the use of the extension is only temporary (and the long term goal is to improve the patient's hair), then the extensions should likely be removed or changed to reduce the chance of long term damage to the hair follicle and the scalp.

Scalp Frontal Fibrosing Alopecia (FFA)

FFA: Scaling Around Hairs

Frontal fibrosing alopecia (FFA) is a type of scarring hair loss that occurs more often in women than men. It causes hair loss along the frontal hairline as well as several other areas including the sides and back of scalp, eyebrows, eyelashes, and body hair.

This picture shows a very typical appearance of the frontal scalp in FFA. There are numerous single hairs, many with scale around those hairs (called perifollicular scaling). A few broken hairs are seen and one hair in the picture is markedly twisted (a phenomenon known as "pili torti"). This is mild scalp redness.

Many treatments are available as we have reviewed together previously. This patient was started on a 5 alpha reductase inhibitor (finasteride, 5 mg) along with pimecrolimus cream (Elidel) and steroid injections. Clobetasol proprionate shampoo (Clobex) will be used weekly and reassessment will be done in 4-6 months.
 

Scaling around hair follicles

Perifollicular Scale

The appearance of white colored scale around hair follicles is common. This can either be concerning - or not concerning. The white scale in the right picture is not concerning and represents a mild scale from normal epidermal turnover. The patient also has androgenetic alopecia. There is only one follicle affected and the scale is not tightly adherent to the hair follicle. When I see this, it catches my attention for just a second and then I move on to assess other scalp features.

The picture on the left shows a pattern of scale which is concerning. When I see hair follicles that look like this I am immediately concerned. This scale is tightly adherent to the follicle and forms a circular shape all around the follicle. It is important to note the underlying redness and it is also important to note that all of the follicles in the photo are just single hairs. Scale that tightly encircles the hair follicle in this manner is known as "perifollicular scale." In this left sided picture, the patient has an underlying scarring alopecia known as lichen planopilaris. Perifollicular scale and perifollicular redness are common in lichen planopilaris (as well as frontal fibrosing alopecia). Scale patterns can change if a patient washes his or her hair within 12 hours of their appointment. Sometimes, in order to better appreciate scaling in patients with challenging diagnoses - I will ask them to refrain from washing the scalp for 24-72 hours. I don't commonly do this but it can be helpful.