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Disease Profile

Pityriasis lichenoides et varioliformis acuta

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

PLEVA; Mucha-Habermann disease

Categories

Skin Diseases

Summary

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.[1][2][3][4]

Symptoms

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.[1][2][3][4]

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.[2][3][4] PLEVA may last for a few weeks to years, and may fluctuate between getting better and worse before going away on its own.[4]

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.[2]

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 pusor 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.[1][2][5]

Cause

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:[1][2][3]

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.[1][2][3]

Diagnosis

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.[1][3]

Treatment

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.[1][3]

If treatment is indicated, there are many different therapies that have been used to treat PLEVA with varying degrees of success. These include:[1][2][3]

Unfortunately, PLEVA may not always respond to treatment and relapses often occur when treatment is discontinued.[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

      Organizations Providing General Support

        Learn more

        These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

        Where to Start

        • The American Osteopathic College of Dermatology provides information on Pityriasis lichenoides et varioliformis acuta.
        • The British Association of Dermatologists provides an informational leaflet on pityriasis lichenoides. Click on the British Association of Dermatologists link to view this leaflet.
        • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
        • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

          In-Depth Information

          • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
          • PubMed is a searchable database of medical literature and lists journal articles that discuss Pityriasis lichenoides et varioliformis acuta. Click on the link to view a sample search on this topic.

            References

            1. Pityriasis lichenoides. DermNet NZ. 1998; https://dermnetnz.org/scaly/pityriasis-lichenoides.html.
            2. Mucha Habermann disease. NORD. 2018; https://rarediseases.org/rare-diseases/mucha-habermann-disease/.
            3. Peter A Klein, MD. Pityriasis Lichenoides. Medscape Reference. October 2014; https://emedicine.medscape.com/article/1099078-overview.
            4. Treat JR. Pityriasis lichenoides et varioliformis acuta (PLEVA). UpToDate. Waltham, MA: UpToDate; https://www.uptodate.com/contents/pityriasis-lichenoides-et-varioliformis-acuta-pleva.
            5. Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert WC. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol. March, 2010; 49(3):257-261. https://www.ncbi.nlm.nih.gov/pubmed/20465660.

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