Appendix
← Clinical Reader
Dermatology

Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)

Pityriasis lichenoides encompasses a group of inflammatory cutaneous disorders known as pityriasis lichenoides chronica (PLC), pityriasis lichenoides et varioliformis acuta (PLEVA), and febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA). PLEVA is a relatively benign cutaneous disorder of unclear origin that affects children and young adults. Patients present with a spontaneous eruption of erythematous macules that evolve into papules, rapidly undergoing hemorrhagic necrosis and

v1 · Reviewed Nov 8, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • febrile ulceronecrotic Mucha-Habermann disease is a life-threatening disease that presents therapeutic challenges.
  • Although rare, febrile ulceronecrotic Mucha-Habermann disease (FUMHD) may follow an existing diagnosis of PLEVA or occur de novo.
  • Patients affected by FUMHD present with necrotic papules that progress to plaques and large coalescent ulcers.
  • Skin ulceration can be extensive and painful, including oral and genital mucosal.
  • Common systemic symptoms include high fever, abdominal pain, diarrhea, central nervous system manifestations, joint pain, respiratory complications, and death.
  • Although rare, PLEVA may evolve into the potentially lethal variant, febrile ulceronecrotic Mucha-Habermann disease.
  • Lymphomatoid papulosis has a significant risk of transforming into a cutaneous malignant lymphoma.
  • A definitive diagnosis of PLEVA requires a thorough history, physical exam, and skin biopsy due to many diseases with similar clinical presentations.
  • Epidermal findings include spongiosis, dyskeratosis, parakeratosis, acanthosis, intraepidermal vesicles, and necrosis.
  • Dermal findings include edema, wedge-shaped superficial and deep dermal lymphohistiocytic inflammatory infiltrate, lymphocyte and erythrocyte extravasation, subepidermal vesicles, and dermal sclerosis.
  • Vascular findings are notable for papillary dermis blood vessel dilation and engorgement with endothelial proliferation, vascular congestion, and erythrocyte extravasation.
  • Dermatoscopic findings for PLEVA have been reported in the literature and include a ring of pinpoint vasculature, brown amorphous areas, white scale, and focal melanin deposition within the stratum corneum.
  • A thorough history and physical exam can have significant value in the diagnosis of PLEVA due to the nature of the disease and uncertainty regarding its etiology.
  • On physical exam, PLEVA manifests as an abrupt eruption of 10-50 erythematous macules that quickly form into inflammatory papules with a fine scale.
  • Although the diagnosis of PLEVA is mainly clinical and aided by dermatoscopy, a histologic evaluation of lesional skin is imperative due to the numerous differential diagnoses with similar presentations.
  • Punch biopsies are recommended to ensure that the epidermis and dermis can be appropriately evaluated.
  • Immunohistochemical studies can aid in confirming the diagnosis and typically show CD8-positive T lymphocytes and negative CD30 stains (to rule out lymphomatoid papulosis).
  • Serological studies are not specific for PLEVA, but findings, including leukocytosis, elevated C-reactive protein, and erythrocyte sedimentation rate, can be seen in FUMHD.
  • Other common/useful non-specific tests to diagnose PLEVA by exclusion include toxoplasma Sabin-Feldman dye, heterophile antibody test, antistreptolysin o titers, throat cultures, HIV screening, and rapid plasma reagin test.
  • Data regarding treatment for the disease is limited, although various retrospective series and case reports recognize systemic antibiotics or phototherapy as first-line therapies in conjunction with corticosteroids.
  • Systemic antihistamines may also offer relief from pruritus.
  • Ultraviolet B light is the most common first-line phototherapy, with no significant difference between narrow and broadband ultraviolet B light.
doxycycline
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Antibiotics are continued until the lesions are resolved for up to 3 months, and in some cases, longer.
minocycline
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Antibiotics are continued until the lesions are resolved for up to 3 months, and in some cases, longer.
erythromycin
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Antibiotics are continued until the lesions are resolved for up to 3 months, and in some cases, longer.
  • If there is no response, phototherapy is considered.
  • Psoralen plus ultraviolet A has been reported as effective in multiple case studies.
  • Ultraviolet A1 has been reported in the literature to aid in the complete resolution of lesions, although it is less accessible and more commonly found in academic centers.
  • Corticosteroids are commonly utilized as adjunctive therapy, and for patients with refractory disease, methotrexate may be an option.
  • Additional therapies for refractory cases include acitretin, dapsone, and cyclosporine.
  • FUMHD may be treated with TNF-a inhibitors such as infliximab due to elevated TNF-a levels reported in a previous pediatric case and success in an adult case.
  • Follow-up monitoring is required in aggressive cases of PLEVA with a waxing and waning course.
  • Patients who choose to receive treatment, such as antibiotics, phototherapy, or corticosteroids, should be monitored closely to assess efficacy.
  • Due to the enigmatic nature of PLEVA, misdiagnosis is a common complication.
  • Lymphomatoid papulosis, for example, is a skin disease that mimics PLEVA, but the separation of the 2 must be emphasized because of differences in prognosis.
  • Pityriasis Lichenoides (PL)-like mycosis fungoides (MF), is a rare form of MF that also appears similarly to PL during the clinical inspection.
  • Although rare, there have been cases reported of CTCL discovered after PLC diagnosis.
  • Concerning outward appearance, the ulceronecrotic lesions in PLEVA may lead to alteration in pigmentation of the skin or heal with varioliform scarring.
  • PLEVA can progress into PLC or present in the more fulminant form, FUMHD.
  • The psychological component of PLEVA must not be overlooked due to its diffuse involvement in exposed skin areas.
  • In most patients, PLEVA is a self-resolving, benign skin disease typically lasting a few weeks.
  • Patients and their families should be educated that the disease is not contagious, and if symptoms worsen, reassessment is necessary.
  • Due to the self-resolving nature of PLEVA, follow-up visits should occur every 3 to 6 months if there is no desire for treatment.
  • Office visits are useful for reevaluating and ensuring no further disease progression or diagnosis change.
  • review
    Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA). StatPearls [Internet] — Fayven Teklehaimanot, Anita Gade, Richard Rubenstein — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov