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Pityriasis lichenoides et varioliformis acuta in Adult
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Pityriasis lichenoides et varioliformis acuta in Adult

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Contributors: Noah Craft MD, PhD, Belinda Tan MD, PhD, Lindy P. Fox MD
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Synopsis

Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease, is a T-cell lymphoproliferative disorder that is characterized by the acute onset of asymptomatic crops of erythematous papules. The papules spontaneously resolve within weeks and recur at a later time. Due to its recurrent nature, PLEVA lesions demonstrate varying stages of evolution and include small ulcers, crusted papules, vesicles, pustules, and varicella-like scarring. PLEVA eruptions are most commonly found in the male pediatric population but can occur in both sexes, in all ages, and in all ethnicities.

While the etiology is not known, the histologic infiltrate is composed of monoclonal CD8+ T lymphocytes. This may help explain its association with lymphomas and cutaneous T-cell lymphoma (CTCL).

Pityriasis lichenoides chronica (PLC) is a related but more chronic form. In contrast to the crusts, vesicles, and pustules seen in PLEVA, PLC takes on a more indolent course and is characterized by crops of scaly erythematous papules that spontaneously regress over months (instead of weeks).

Variants of PLEVA include those with systemic manifestations such as fever, generalized lymphadenopathy, malaise, arthralgias, and arthritis. A severe variant, pityriasis lichenoides, ulceronecrotic, hyperacute (PLUH), is defined by more severe cutaneous and systemic findings. Large lesions with necrotic centers, ulcers, and diffuse purpuric papules can occur. Higher fevers, myalgias, arthralgias, and central nervous system and gastrointestinal symptoms have been described. PLUH carries a 25% mortality rate and is considered a dermatologic emergency.

PLEVA is generally viewed as a benign lymphoproliferative disorder that lasts from 1-3 years, depending on the distribution of lesions. However, there are case reports of progression to CTCL. No guidelines have been established for monitoring this possible progression.

Codes

ICD10CM:
L41.0 – Pityriasis lichenoides et varioliformis acuta

SNOMEDCT:
86487001 – Acute lichenoid pityriasis

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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 rheumatoid factor (RF), antinuclear antibodies (ANA), anti-ds DNA, antineutrophil cytoplasmic antibody (ANCA), cryoglobulins, and 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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References

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Last Updated: 08/08/2019
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Pityriasis lichenoides et varioliformis acuta in Adult
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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
Many erythematous papules, some with overlying hemorrhagic crusting, on the buttocks.
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