Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Pityriasis lichenoides et varioliformis acuta


Other Names for this Disease
  • Mucha-Habermann disease
  • PLEVA
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have a 7-year-old patient diagnosed with PLEVA by skin biopsy. He has a chronic and relapsing course and we are trying to find more information for the family regarding this condition, its outcomes, and long-term complications.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What are the signs and symptoms of pityriasis lichenoides et varioliformis acuta?

Pityriasis lichenoides et varioliformis acuta (PLEVA) is the acute form of a skin condition called pityriasis lichenoides. It is characterized by the sudden onset of red patches that quickly develop into scaling papules. These papules may become filled with blood and/or pus or erode into crusted red-brown spots. People may also experience burning and itching of the affected area. Scarring and/or temporary discoloration of the skin may be present after the lesions have healed.[1][2][3]

Although PLEVA can affect almost any part of the body, it most commonly develops on the trunk and/or limbs (arms/legs). Affected people may have a few to more than one hundred papules. The skin abnormalities generally resolve without treatment in a few weeks to a few months; however, some people experience episodes of the condition on and off for years.[2][3]

Aside from the skin findings, most affected people do not experience any additional signs and symptoms. However, some may experience fever, headaches, joint pain and swelling of nearby lymph nodes.[2]

Febrile Ulceronecrotic Mucha-Haberman Disease is a rare and severe variant of PLEVA that is associated with unique signs and symptoms.[1][2] For more information on this condition, please click here.
Last updated: 2/2/2015

How often does pityriasis lichenoides et varioliformis acuta turn into something more serious?

Pityriasis lichenoides et varioliformis acuta (PLEVA) may evolve into cutaneous lymphoma in less than 2% of cases.

Rarely, a severe variant of PLEVA called febrile ulceronecrotic Mucha-Habermann disease (FUMHD) develops. FUMHD often begins as PLEVA, but then rapidly and suddenly progresses to large, destructive ulcers. There may be fever and extensive, painful loss of skin tissue. Patients may develop complications such as interstitial pneumonitis, abdominal pain, central nervous system involvement, bacteremia, and sepsis.[3] For more information on this condition, please click here.
Last updated: 2/3/2015

What is the long-term outlook for people with pityriasis lichenoides et varioliformis acuta?

The long-term outlook (prognosis) for people with pityriasis lichenoides et varioliformis acuta (PLEVA) varies but is generally good. Most cases tend to resolve on their own in several weeks to several months. One study of 22 children found that the average duration of PLEVA was approximately 1.6 months; however, the duration may be longer in adults. Some people may experience episodes of the condition on and off for years.[3]

The prognosis tends to be worse in people who develop a rare variant of PLEVA called Febrile Ulceronecrotic Mucha-Haberman Disease.[3] For more information on this condition, please click here.
Last updated: 2/3/2014

References
Other Names for this Disease
  • Mucha-Habermann disease
  • PLEVA
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.