What is Graham-Little-Piccardi-Lasseur Syndrome?
Graham-Little-Piccardi-Lasseur syndrome, or GLPLS, is a rare disorder linked to a condition called lichen planopilaris, which affects the hair follicles. It was first extensively studied by two doctors, Piccardi in 1913 and Ernst Graham Little in 1915, along with their patient named Lasseur.
Lichen planopilaris can present itself in three different forms, including GLPLS, frontal fibrosing alopecia, and classic lichen planopilaris. Classic lichen planopilaris often leads to patchy hair loss, due to chronic inflammation. Frontal fibrosing alopecia results in a band-like pattern of hair loss on the front and sides of the scalp. GLPLS causes patchy hair loss on the scalp, hairless areas in the armpits and around the genitals, and rough, thickened skin on the trunk and limbs. The order in which these symptoms occur can vary, but it usually starts with rough, thickened skin before leading to hair loss.
GLPLS makes up a small portion of all lichen planopilaris cases and primarily impacts white women between 30 and 70 years old. Several factors may contribute to its development, including a hepatitis B vaccination, hormone imbalances, vitamin A deficiency, certain genetic markers, and mental health issues. Treatment for GLPLS aims to decrease the immune response to avoid scars forming around hair follicles. However, effective treatments are few, although interferon-based therapies show promise. Often, complete patient recovery can be challenging, indicating the need for better preventive strategies to avoid long-term health problems.
Let’s talk about how our hair works. Hair follicles, or tiny pockets from which our hair grows, consist of two parts. The upper part extends from the skin’s surface to the oil-producing sebaceous duct. The lower part contains the ‘stem’ and the ‘bulb’ of the hair, where keratinization, or hardening of the hair and skin, occurs. The bulb contains a range of cells necessary for hair growth and the central capillary supplying blood and nutrients.
Hair can be categorized into three types: lanugo, vellus, and terminal. Lanugo hair is fine and covers the fetus, usually disappearing after birth. Vellus hair is short and fine, with a diameter smaller than 0.03 mm, and its bulb is located in the upper dermis. Terminal hair, longer and thicker, is found in places sensitive to androgens (male hormones), such as the scalp, beard, chest, armpits, and pubic area, and also in androgen-independent areas like eyebrows and eyelashes. Vellus hair, however, is not influenced by androgens.
About 85% to 100% of scalp hairs are typically growing in the anagen phase, which lasts 2 to 7 years. Around 1% of scalp hairs exist in the catagen phase, a short phase of 2 to 3 weeks before shedding. 0% to 15% of hairs are in telogen, the resting phase where old hairs fall out and new ones begin to grow.
What Causes Graham-Little-Piccardi-Lasseur Syndrome?
The cause of GLPLS, a skin condition, is mostly unknown but many believe it happens because of an autoimmune process where the body’s own T-cells mistakenly attack body tissues. This means that the body’s own defense system accidentally harms its own tissues.
Conditions like lichen planopilaris, characterized by inflammation and altered integrin expression, or a change in the proteins that help cells stick to each other, could contribute to GLPLS. Inflammation and these protein changes can lead to cicatricial alopecia, a type of hair loss where the hair roots are damaged. This can be observed when hair pulls out easily during a test.
One study reported a strong immune response to a protein found at the center of our cells during cell division, named “INCENP”. This suggests that issues with cell division might play a part. While more research is needed to understand how often these INCENP immune responses happen in GLPLS patients, it’s the first time an antibody has been related to GLPLS.
Recently, there have also been reports of GLPLS being seen across three generations of a family, suggesting that there might be a genetic component.
Risk Factors and Frequency for Graham-Little-Piccardi-Lasseur Syndrome
GLPLS, a variant of lichen planopilaris, mostly strikes white women between the ages of 30 and 70. However, it represents just a small percentage of overall cases. In a research study of 80 people with lichen planopilaris, only one case was diagnosed as GLPLS. Several factors seem to be associated with this condition, such as receiving the hepatitis B vaccine, having an androgen insensitivity syndrome, a mother and daughter being positive for HLA DR-1, a deficiency of vitamin A, hormonal problems, and mental health issues.
Signs and Symptoms of Graham-Little-Piccardi-Lasseur Syndrome
Graham-Little Piccardi Lassueur syndrome (GLPLS) is a condition that affects the hair and skin. It’s diagnosed through a detailed medical history and physical examination. People with this condition usually have a distinctive pattern of hair loss with bald patches and scarring on the scalp. Hair loss might also occur in the armpit and genital areas without scarring. Another prominent feature of GLPLS is bumpy skin, especially on the trunk and limbs, with bumps turning into plaques over time. Symptoms usually develop gradually, with skin changes appearing first, followed by hair loss.
Additionally, details about how much hair is lost, any emotional stress the patient is experiencing, or any hormonal imbalances they may have, might aid in ruling out other causes of hair loss.
In a physical examination, such symptoms can be confirmed by inspecting the entire body. Key signs to look out for include hair loss on the scalp with proof of scarring and hair loss in the armpits and genital region without apparent scarring signs. The patient will likely show patches of roughened, red skin around hair follicles on the trunk and extremities. When testing the strength of hair follicles by pulling them, they are typically weak and easily come out in people with GLPLS. Viewing the affected areas under a dermoscope, a device used for examining skin conditions, can reveal redness and flaking of skin around hair follicles in the areas of hair loss.
Testing for Graham-Little-Piccardi-Lasseur Syndrome
The main ways doctors diagnose Graham-Little Piccardi Lasseur Syndrome (GLPLS), a rare condition that affects the hair and skin, are through a physical examination and a skin biopsy. A skin biopsy involves taking a small sample of skin to be examined under a microscope.
When this skin sample is studied, features of a condition called lichen planopilaris show up. This shows that inflammation, or swelling, around the hair follicles is leading to the destruction of important skin structures including the hair follicles and sebaceous glands (glands which produce oils for the skin). If GLPLS is in its late stages, the biopsy might show damage to the arrector pili muscles, the tiny muscles that cause goosebumps.
In a physical examination, the doctor usually finds red bumps around the hair follicles that are between 1 to 4 mm in size, hair loss in the areas around the genitals and under the arms that does not cause scars, and patchy hair loss on the scalp that does cause scars. Additionally, hard bumps, which might be itchy, often appear on the trunk, thighs, arms, and wrists. These bumps may come before the hair loss starts on the scalp, but the order of these symptoms can vary from person to person.
Treatment Options for Graham-Little-Piccardi-Lasseur Syndrome
Managing GLPLS, a kind of skin inflammation, can be quite challenging because there isn’t one widely recognized treatment. The main goal of treatment is to limit the immune system’s response before it leads to changes in hair follicles (small sacs in the skin where hair grows).
Recent research is looking into something called JAK inhibition, using a drug called tofacitinib. This is thought to help control the immune response by reducing unnecessary inflammation in the hair follicles. In a small set of 8 people with GLPLS, it was found that taking tofacitinib twice a day led to improvements in the skin condition by 30-94%.
There was another method of management described by Zegarska and others that included a treatment plan using a medication called prednisone, as well as psoralen (a medication that makes the skin more responsive to light) and ultraviolet light A. This approach moderately improved some signs of the disease, particularly follicular hyperkeratosis, which is excessive skin growth around hair follicles. This method was not as successful in reducing hair loss in specific areas like the scalp, underarms, and the area around the genitals.
Other treatments used for GLPLS may include various medications applied directly to the skin or taken orally, like intralesional glucocorticoids, topical glucocorticoids, systemic glucocorticoids, cyclosporine, tacrolimus ointment, and systemic retinoids. While these treatments can often help with skin redness and inflammation around hair follicles, they may not significantly affect hair loss in certain areas of the body. More recently, there have been documented cases of successful management of GLPLS using a light therapy known as narrow-band UVB phototherapy.
What else can Graham-Little-Piccardi-Lasseur Syndrome be?
When trying to figure out if someone has GLPLS, doctors might also consider these conditions that can have similar symptoms:
- Classical lichen planopilaris
- Pseudopelade of Brocq
- Frontal fibrosing alopecia
- Lichen spinulosus
- Androgen insensitivity syndrome
- Discoid lupus erythematosus
- Secondary syphilis
- Addison disease
These illnesses often show up with skin issues and scalp problems that look a lot like the symptoms of GLPLS. A detailed check-up and the right tests can help doctors decide how best to treat the issue.
What to expect with Graham-Little-Piccardi-Lasseur Syndrome
The progression of GLPLS, a condition causing hair loss, typically progresses slowly over time. Although treatment can help reduce some symptoms, patients often find it hard to completely get rid of the hair loss problems. The data on how long this disease lasts in average is limited, but we know that in some cases, it can continue for more than 20 years. For example, a study by Zegarska and others reported that the disease lasted anywhere between 6 months to 10 years in the cases they studied.
Proper medical management can immensely help improve the patient’s condition, especially in reducing hyperkeratotic papules- condition characterized by rough patches of skin, usually found on the trunk and the arms or legs.
Possible Complications When Diagnosed with Graham-Little-Piccardi-Lasseur Syndrome
GLPLS, or general lichen planopilaris, can lead to substantial emotional stress as it significantly affects an individual’s appearance. Despite this, the condition doesn’t show other systemic symptoms or signs in the body outside of the affected skin. Future investigations into INCENP’s role, especially in mitotic function, could potentially reveal linked health issues, but so far, none are identified. The primary lasting issue with GLPLS is follicular scarring. This is due to inflammation in particular areas, which results in irreversible damage to pilosebaceous units, especially in the scalp.
Common Issues:
- Emotional distress due to altered appearance
- No systemic symptoms
- Potential discovery of linked health conditions in future research
- Follicular scarring due to inflammation
- Permanent damage to pilosebaceous units in the scalp
Preventing Graham-Little-Piccardi-Lasseur Syndrome
If you have GLPLS, it’s important to have a detailed understanding of what to expect in the future and what treatment options are available. This will help you have a realistic outlook. Some people choose not to undergo treatment because the current therapies often do not solve hair loss related to the condition. Even though treatments can’t fix the irreversible damage, starting treatment early could potentially slow down the advance of non-scarring hair loss, scarring hair loss on the scalp, and development of hard skin bumps.
Understanding the purpose of your treatment can help you stick to it and can strengthen your relationship with your doctor. In simple terms, non-scarring hair loss means that your hair might fall out but the scalp is not damaged and hair can potentially regrow. Scarring hair loss, on the other hand, means that the hair loss is accompanied by damage to the scalp, making it hard for the hair to grow back. Hard skin bumps refer to small, rough skin growths that can occur.
