What is Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)?
Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha-Habermann disease, is a rare skin rash. It is recognized by its widespread red-brown spots in different stages with a flaky, mica-like (a mineral that looks like thin sheets) shell covering the older sores. These spots may develop into blisters, pustules (small pus-filled bumps), and open sores. These lesions might cause an itching or a burning feeling. PLEVA typically affects the chest and the closer parts of the arms and legs, particularly in the bending areas like the elbow or knee. This skin condition tends to come and go, sometimes over several years. You can see as an example an image of an open PLEVA sore on the arm.
What Causes Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)?
The exact cause of PLEVA, a skin condition, is still unknown. But, there are several theories about what might cause it. One theory is that it might be an unusual response of the body’s immune system to infections. Another theory is that it might be a reaction to certain medications. Yet another theory is that it could be related to an increase in lymph cells, the type of white blood cells that fight infections.
In support of these theories, there have been instances where PLEVA occurred after infections with different types of viruses and bacteria, including Epstein-Barr virus, HIV, varicella-zoster virus, Toxoplasma gondii, strep bacteria, and herpes simplex virus type 2.
There have also been instances where PLEVA has occurred after receiving certain vaccines, including the human papillomavirus, influenza, tetanus, measles, mumps, and rubella (MMR) vaccines.
Because PLEVA looks similar to lymphomatoid papulosis, a skin disease that can sometimes progress to a type of cancer called lymphoma, it’s important to consider the theory that PLEVA could possibly be related to an increase in lymph cells, a condition known as lymphoproliferation or T cell dyscrasia.
PLEVA has also been known to develop after taking certain medications, including atezolizumab, pembrolizumab, and a topical medication called diphenylcyclopropenone.
Risk Factors and Frequency for Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
Pityriasis lichenoides et varioliformis acuta (PLEVA) is a disease for which we don’t yet fully understand the number of cases, the commonness, or the risk factors. It can affect anyone, regardless of race or gender, but is most frequently seen in children and young adults. Although it affects both genders, it’s slightly more common in males.
Signs and Symptoms of Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
Diagnosing PLEVA (Pityriasis Lichenoides Et Varioliformis Acuta), a skin disease with unclear causes, often involves asking the patient about recent vaccines, illnesses, and medications. On physical examination, you may find the sudden appearance of numerous reddish spots (macules) that quickly turn into small, inflamed bumplike lesions (papules), accompanied by fine scale. As the rash develops, the scale might remain attached centrally but lifts at the edges. In the course of the disease’s progression, the papules might change into long-term lesions or undergo a breakdown and become ulcerated, leading to the appearance of red-brown crusts and potential changes in skin color.
- PLEVA can also produce no symptoms or cause a burning feeling and itchiness.
- These various lesions generally appear on the trunk and inner surfaces of the limbs, but they may spread elsewhere.
- PLEVA often comes and goes, and can last several weeks to years before it goes away completely.
- The disease can, in severe cases, lead to febrile ulceronecrotic Mucha-Habermann disease (FUMHD), which is more serious and potentially deadly.
For patients with FUMHD, the papules become necrotic and proceed to form large, merged ulcers. These skin ulcers can be widespread and painful, and may affect the oral and genital mucosa. Systemic symptoms could include high fever, stomach pain, diarrhea, nervous system symptoms, joint pain, and respiratory issues, and in extreme cases, it can cause death.
Testing for Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
If you’re suspected to have PLEVA, a skin condition characterized by itchy, scaling rashes, your doctor will conduct an examination and use a tool called a dermatoscope to look at your skin more closely. However, confirming the diagnosis of PLEVA usually requires taking a small sample of your skin (a procedure known as a punch biopsy) for further examination in a lab.
In the lab, they’ll conduct a special type of testing called immunohistochemical studies. These tests can reveal whether there are specific kinds of immune cells present in the skin sample. If there are numerous CD8-positive T lymphocytes (a type of white blood cell) and negative CD30 stains (which rules out a related skin disease), it helps to confirm a PLEVA diagnosis.
Other general blood tests can provide additional information. Although they are not specific for PLEVA, findings such as increased white blood cells (leukocytosis), higher levels of C-reactive protein (a sign of inflammation in the body), and an elevated erythrocyte sedimentation rate (which measures how fast red blood cells settle at the bottom of a tube, typically higher in conditions causing inflammation or tissue damage) can suggest PLEVA or the more severe variant, FUMHD.
Moreover, your doctor may suggest other tests such as toxoplasma Sabin-Feldman dye, heterophile antibody test, antistreptolysin o titers, throat cultures, HIV screening, and rapid plasma reagin test. These tests can help doctors diagnose PLEVA by ruling out other conditions with similar symptoms.
Treatment Options for Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
While PLEVA, a benign skin condition, doesn’t necessarily need treatment, starting medication can speed up recovery and help to relieve symptoms if the patient is uncomfortable. There’s a limited amount of information regarding treatment methods for PLEVA. However, some studies and patient case reports have recommended the use of systemic antibiotics or phototherapy (light therapy) as the first step, along with corticosteroids, which are medications used to reduce inflammation. Systemic antihistamines, which are drugs often used to treat allergies, may also help to relieve itching.
In severe cases of PLEVA that exhibit large ulcers, caring for the wound locally may be beneficial. Doctors may need to routinely monitor patients with aggressive forms of PLEVA that persist or recur over time. Antibiotics that are also known to reduce inflammation, like doxycycline and minocycline (or erythromycin for children), are often used. The patient may have to continue taking these antibiotics until the skin lesions have completely healed, which could take up to three months or more in some cases. If there’s no improvement, doctors may consider using phototherapy.
The most commonly used type of phototherapy for PLEVA is ultraviolet B light. It’s important to note that the long-term effects of phototherapy aren’t known. However, in children who underwent this treatment, no immediate side effects were reported. For adults, the chosen therapy would usually depend on their preference and other health issues they may have.
Aside from these treatment options, corticosteroids are also commonly used alongside other treatments. If a patient’s condition doesn’t improve with these treatments, doctors might consider using a medication called methotrexate. Some additional treatments for stubborn cases could include acitretin, dapsone, and cyclosporine.
In some instances, PLEVA may become severe and turn into a life-threatening condition called febrile ulceronecrotic Mucha-Habermann disease, or FUMHD. This condition can present many challenges for doctors in finding an effective treatment since each patient may respond differently to the same treatment methods. In such cases, medications that inhibit TNF-a (a protein in the body that triggers inflammation), such as infliximab, might be used. There’s a need for more thorough studies, like randomized control trials, to fully grasp the benefits of these suggested treatments.
What else can Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA) be?
When a doctor is trying to diagnose a skin condition called PLEVA, they might consider other conditions that can cause similar symptoms. These include:
- Langerhans cell histiocytosis
- Gianotti-Crost syndrome
- General reaction to insect bites
- Widespread herpes simplex virus
- Varicella Zoster virus (the virus that causes chickenpox and shingles)
- Cutaneous small vessel vasculitis (inflammation of the blood vessels in the skin)
- Insect bite
- Secondary syphilis
- Polymorphous light reaction (a skin reaction to sunlight)
- Generalized folliculitis (inflammation of the hair follicles)
- Pityriasis lichenoides chronica (a rare skin disorder)
- Lymphomatoid papulosis (a rare skin disorder)
- Lichen planus (a condition that can cause swelling and irritation in the skin, hair, nails and mucous membranes)
- Hypopigmented mycosis fungoides (a type of skin lymphoma)
- Papular eczematous dermatitis (a type of skin inflammation)
- Pityriasis rosea (a skin rash)
- Guttate psoriasis (a type of psoriasis that appears as small, salmon-pink bumps on the skin)
- Urticaria pigmentosa (a condition that causes red or brown itchy bumps on the skin)
- Exanthematous drug eruption (a widespread skin rash caused by certain medications)
A thorough check-up and appropriate tests are important for the doctor to make an accurate diagnosis.
What to expect with Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
Pityriasis lichenoides et varioliformis acuta, or PLEVA, is a rare skin condition that can, in rare cases, progress into a potentially life-threatening variant known as febrile ulceronecrotic Mucha-Habermann disease. It has also been associated in some instances with a type of skin cancer known as cutaneous T-cell lymphoma. However, it’s important to note that these scenarios are uncommon, and the general outlook for people with PLEVA is usually very good. This is because it’s generally mild in nature and the related conditions are rare.
Possible Complications When Diagnosed with Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
PLEVA, or Pityriasis Lichenoides et Varioliformis Acuta, is a skin condition that can be tricky to diagnose correctly since it shares similarities with other skin diseases. For instance, it can be confused with lymphomatoid papulosis, another skin condition. It’s essential to make a clear distinction between them as lymphomatoid papulosis can eventually develop into a dangerous skin cancer known as cutaneous malignant lymphoma.
Another condition that can be confused with PLEVA is Pityriasis Lichenoides (PL)-like mycosis fungoides (MF), a rare form of MF that seems similar to PL. Even though it has some features like Pautrier’s microabscess and others, which are typically seen in regular MF, it is vital to look at the tissue under a microscope when considering these conditions to distinguish LP and PL-like MF from different types of PL.
In some cases, further investigation may be needed if PLEVA becomes severe or isn’t responding to treatment. Rarely, CTCL, a type of skin cancer, has been found after a diagnosis of PLEVA. Also, the skin changes due to PLEVA, such as color changes or scarring, can potentially affect a person’s mental health, especially since it often affects exposed areas of the skin.
Summary:
- PLEVA can be mistaken for other similar looking skin conditions like lymphomatoid papulosis and Pityriasis Lichenoides (PL)-like mycosis fungoides (MF).
- It’s crucial to accurately diagnose these conditions due to their different outcomes and potential risks.
- More extensive studies may be needed if PLEVA gets severe or does not respond to treatment. Occasionally, a type of skin cancer named CTCL is found after a PLEVA diagnosis.
- The changes to skin color and scarring resulting from PLEVA can impact mental health as the condition often affects visible areas of skin.
Preventing Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
PLEVA, or muchilaniform lymphocytic vasculitis associated with skin lesions, is a benign disease of the skin that usually goes away on its own in a few weeks. It’s important for patients and their families to know that it can’t be passed from person to person. If the symptoms get worse, a doctor should reassess the patient’s condition.
