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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
  • PLEVA
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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 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 complications may be associated with pityriasis lichenoides et varioliformis acuta (PLEVA)?

Those with PLEVA are often left with scarring. A rare severe variant of PLEVA (febrile ulceronecrotic Mucha-Habermann disease - FUMHD) is characterized by the rapid development of numerous black or necrotic bumps (papules) on the skin, high fever and joint pain. The skin lesions may grow and spread rapidly, eventually combining into extremely painful ulcers and blisters. These lesions tend to be larger than those associated with PLEVA, may bleed, become infected, and scar upon healing. Those with FUMHD may also develop other complications such as gastrointestinal abnormalities (e.g., diarrhea, abdominal pain, and malabsorption), enlargement of the spleen, inflammation of the lungs (interstitial pneumonitis), central nervous system abnormalities, bacteremia, and/or sepsis. Very rarely, those with FUMHD can develop cutaneous T-cell lymphoma. Because lesions associated with FUMHD can become cancerous, some physicians have suggested that a follow-up biopsy should be performed on lesions that last longer than 1 year and are refractory to treatment.[3][4]
Last updated: 1/3/2012

What is the prognosis for those with pityriasis lichenoides et varioliformis acuta (PLEVA)?

There is no clear consensus about the exact duration of the disease. Most cases tend to resolve over time. One report of 22 children found that the mean duration for PLEVA to complete resolution was 1.6 months. In most cases, the disease resolves spontaneously over time; but in some people, the disease may remit and recur over years. The disease duration may be longer in adults.[3]
Last updated: 1/3/2012

References
  • 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.
  • 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.
  • Klein PA. Pityriasis Lichenoides. eMedicine. July 2010; http://emedicine.medscape.com/article/1099078-overview. Accessed 1/3/2012.
  • 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.