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Pityriasis lichenoides et varioliformis acuta - Skin in Child
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Pityriasis lichenoides et varioliformis acuta - Skin in Child

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Contributors: Belinda Tan MD, PhD, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
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Synopsis

Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease, is an idiopathic cutaneous disease within the spectrum of diseases known as pityriasis lichenoides. It is clinically characterized by recurrent crops of papules that become necrotic and then heal with pigmentary change or minor scarring. Febrile ulceronecrotic Mucha-Habermann disease and pityriasis lichenoides chronica are the other entities within the pityriasis lichenoides group. They are characterized by large coalescent, ulcerative lesions with systemic symptoms or an indolent course with lesions that resolve without necrosis, respectively. PLEVA has a peak onset at ages 5 and 10 years and may last weeks to years before spontaneously resolving. Although generally considered a benign disease, rare case reports have documented possible transformation to T-cell lymphomas.

Codes

ICD10CM:
L41.0 – Pityriasis lichenoides et varioliformis acuta

SNOMEDCT:
86487001 – pityriasis lichenoides et varioliformis acuta

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Skin biopsy will aid in the diagnosis. The key entities to rule out in the differential diagnosis include lymphomatoid papulosis, small vessel vasculitis, varicella, and arthropod reactions:
  • Lymphomatoid papulosis – Predominantly CD30+ cells in the infiltrate, in older patients, characterized by more nodular lesions, and active lesions do not spontaneously resolve as quickly as PLEVA.
  • Vasculitis – Check serologies for RF, ANA, anti-ds DNA, ANCA, cryoglobulins. C3, C4 levels. Lesions are mostly purpuric and more monomorphous.
  • Varicella – Prodrome of mild fever, malaise, and myalgia followed by pruritic erythematous papules. Lesions are pruritic. Recurrent eruptions are not a feature of varicella.
  • Arthropod bites
Other entities within the differential include:

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Therapy

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References

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Last Updated: 06/15/2018
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Pityriasis lichenoides et varioliformis acuta - Skin in Child
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Pityriasis lichenoides et varioliformis acuta : Scattered many, Smooth papules, Maculopapular erythema, Scaly papules
Clinical image of Pityriasis lichenoides et varioliformis acuta
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