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Pityriasis lichenoides et varioliformis acuta


Other Names for this Disease

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

Overview

What is pityriasis lichenoides et varioliformis acuta (PLEVA)?

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


What causes pityriasis lichenoides et varioliformis acuta (PLEVA)?

How might pityriasis lichenoides et varioliformis acuta (PLEVA) be treated?

What is pityriasis lichenoides et varioliformis acuta (PLEVA)?

Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare skin disorder that is characterized as a more severe form of pityriasis lichenoides. Signs and symptoms usually begin with itchy, burning skin lesions that ulcerate, breakdown, form open sores, then form a red-brown crust. Individuals with PLEVA may also experience a low-grade fever, headache, malaise (a vague feeling of bodily discomfort), and arthralgias (severe joint pain); these symptoms may occasionally precede or accompany the skin findings. The underlying cause of pityriasis lichenoides and PLEVA is unknown. Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of PLEVA.[1][2]
Last updated: 1/3/2012

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

PLEVA is a skin disorder that is a more severe form of pityriasis lichenoides. The first symptom of the disease is usually red patches that quickly evolve into papules. The papules erupt into lesions that are filled with puss and blood, which often heals with scarring and changes in skin coloring (pigmentation), either becoming darker or lighter. The skin eruption may cause burning or itching and can look like chicken pox.  The face is rarely affected, but the spots are usually scattered on the trunk and limbs. Those with PLEVA may also experience a low-grade fever, headache, malaise (a vague feeling of bodily discomfort), and severe joint pain; these symptoms may occasionally precede or accompany the skin findings.[1][2]
Last updated: 1/3/2012

What causes pityriasis lichenoides et varioliformis acuta (PLEVA)?

The exact cause of pityriasis lichenoides et varioliformis acuta (PLEVA) is unknown.[3][4][5]  However, three major theories regarding the cause have been proposed. These include:[3]

  • An inflammatory reaction triggered by infectious agents
  • A relatively benign form of T-cell lymphoproliferative disorder
  • An immune-complex-mediated hypersensitivity vasculitis

Some of the infections that have been associated with PLEVA include:[3][4]

Last updated: 1/3/2012

How might pityriasis lichenoides et varioliformis acuta (PLEVA) be treated?

Many treatments have been reported for PLEVA, although their effectiveness varies among people with this condition. Depending on the severity of PLEVA, treatment may include increased sun exposure, topical steroids, topical immunomodulators (such as tacrolimus or pimecrolimus), oral antibiotics (such as erythromycin or tetracycline), phototherapy, systemic steroidsmethotrexate, acitretin, dapsone, ciclosporin, or etanercept. PLEVA may not always respond to treatment and relapses often occur when treatment is discontinued.[3][6]
Last updated: 5/2/2012

References
  1. Ersoy-Evans S, Fernanda Greco M, Mancini AJ, Subasi N, Paller AS.  Pityriasis lichenoides in childhood: A retrospective review of 124 patients.  J Am Acad Dermatol. Feb 2007; 56(2): 205-210. http://www.ncbi.nlm.nih.gov/pubmed/17097385.
  2. Bowers S, Warshaw EM.  Pityriasis lichenoides and its subtypes.  J Am Acad Dermatol. Oct 2006; 55(4): 557-572. http://www.ncbi.nlm.nih.gov/pubmed/17010734.
  3. Pityriasis lichenoides. New Zealand Dermatological Society Web site. 2011; http://dermnetnz.org/scaly/pityriasis-lichenoides.html. Accessed 1/3/2012.
  4. Klein PA. Pityriasis Lichenoides. eMedicine. July 2010; http://emedicine.medscape.com/article/1099078-overview. Accessed 1/3/2012.
  5. Paller AS. Mucha Habermann Disease. National Organization for Rare Disorders (NORD). 2009; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/752/viewAbstract. Accessed 1/3/2012.
  6. Nikkels AF, Gillard P, Piérard GE. Etanercept in therapy multiresistant overlapping pityriasis lichenoides. J Drugs Dermatol. 2008 Oct;7(10):990-2. http://www.ncbi.nlm.nih.gov/pubmed/19112768 .


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