What is the definition of Pityriasis Lichenoides Et Varioliformis Acuta?
Pityriasis lichenoides et varioliformis acuta (PLEVA) is the acute form of an inflammatory skin condition called pityriasis lichenoides. People with PLEVA may develop a few to more than one hundred scaling papules which may become filled with blood and/or pus, or erode into crusted red-brown spots. Papules may itch or burn, and some people may experience fever or joint pain. Although PLEVA may occur at any age (including infancy), it most commonly affects children and young adults. A skin biopsy is needed to confirm the diagnosis. The exact underlying cause is unknown, but some scientists suspect that it may occur due to an exaggerated immune response to an infection or an overproduction of certain white blood cells (a lymphoproliferative disorder). If treatment is necessary, recommended therapies may include oral antibiotics, sun exposure, topical steroids, immunomodulators (medications used to help regulate or normalize the immune system), phototherapy and/or systemic steroids. PLEVA may last for a few weeks to years, and may fluctuate between getting better and worse before going away on its own.
What are the alternative names for Pityriasis Lichenoides Et Varioliformis Acuta?
- Mucha-Habermann disease
What are the causes for Pityriasis Lichenoides Et Varioliformis Acuta?
The exact underlying cause of pityriasis lichenoides et varioliformis acuta (PLEVA) is unknown. Some scientists suspect that it may occur due to an exaggerated immune response or hypersensitivity to an infection. Some of the infections that have been associated with PLEVA include:
- Toxoplasma gondii
- Epstein-Barr virus
- Parvovirus (fifth disease)
- Staphylococcus aureus
- Group A beta-haemolytic streptococci
Others scientists think the condition may be a benign lymphoproliferative disorder. These conditions are characterized by an overproduction of certain white blood cells (lymphocytes) which can result in tissue and organ damage.
What are the symptoms for Pityriasis Lichenoides Et Varioliformis Acuta?
PLEVA is characterized by the sudden onset of red patches that quickly develop into scaling papules. Some people report having had infectious symptoms (such as a respiratory infection) prior to the onset. The papules may become filled with blood and/or pus or erode into crusted red-brown spots. People with PLEVA may also experience burning and itching of the affected areas. Scarring and/or temporary discoloration of the skin may be present after the lesions have healed.
Although PLEVA can affect almost any part of the body, it most commonly develops on the trunk, arms, and/or legs. The number of papules may range from a few to over one hundred. Individual papules often resolve after a few weeks, but new ones often develop as older ones resolve, resulting in ongoing symptoms. PLEVA may last for a few weeks to years, and may fluctuate between getting better and worse before going away on its own.
Aside from the skin features, most people do not experience additional signs and symptoms. However, some people experience fever, headaches, joint pain and/or swelling of nearby lymph nodes.
There is a rare subtype of PLEVA called febrile ulceronecrotic Mucha-Haberman disease (FUMHD) in which papules rapidly develop into painful, large, crusted ulcers and pus- or blood-filled blisters, which may become infected. People with FUMHD may also have symptoms such as high fever, sore throat, diarrhea, abdominal pain, neurologic abnormalities, lung disease, and other symptoms. In some cases, FUMHD can be fatal.
What are the current treatments for Pityriasis Lichenoides Et Varioliformis Acuta?
Pityriasis lichenoides et varioliformis acuta (PLEVA) often resolves on its own within several weeks to several months. Depending on the severity of the condition and the symptoms present, treatment may not be necessary.
If treatment is indicated, there are many different therapies that have been used to treat PLEVA with varying degrees of success. These include:
- Oral antibiotics
- Sun exposure
- Topical steroids
- Immunomodulators (medications used to help regulate or normalize the immune system)
- Systemic steroids
Unfortunately, PLEVA may not always respond to treatment and relapses often occur when treatment is discontinued.
What is the outlook (prognosis) for Pityriasis Lichenoides Et Varioliformis Acuta?
The duration and severity of PLEVA varies from person to person and cannot be predicted. Most cases resolve over time, but it may last for weeks, months, or years. Some people may experience alternating periods of time when symptoms get better or worse for years. The condition may last longer in adults than in children.
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a rare, severe type of PLEVA, can be associated with serious complications and can be fatal, particularly in adults.
How is Pityriasis Lichenoides Et Varioliformis Acuta diagnosed?
A diagnosis of pityriasis lichenoides et varioliformis acuta is often suspected based on characteristic signs and symptoms. A skin biopsy can be used to confirm the diagnosis. Additional laboratory testing may be ordered to investigate a possible cause such as an associated infection.