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Genetic and Rare Diseases Information Center (GARD)

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Pityriasis lichenoides et varioliformis acuta

Other Names for this Disease
  • Mucha-Habermann disease
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Your Question

What is the incidence of pityriasis lichenoides et varioliformis acuta (PLEVA) in the United States? How often does PLEVA turn into something more serious?

Our Answer

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What is the incidence of pityriasis lichenoides et varioliformis acuta (PLEVA) in the United States?

The incidence of PLEVA in the United States has not been reported.[1]
Last updated: 8/6/2010

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

Occasional cases (<2%) of PLEVA have been reported to evolve into cutaneous lymphoma. 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 as well as secondary infection of the ulcers. Patients may develop complications such as interstitial pneumonitis, abdominal pain, malabsorption, central nervous system involvement, bacteremia, sepsis, and rheumatic manifestations.[1]
Last updated: 10/22/2010