Pityriasis Lichenoides et Varioliformis Acuta / Mucha-Habermann disease

Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha-Habermann disease, is a rare inflammatory skin disorder characterized by the sudden eruption of small, red-brown papules that may evolve into vesicles, pustules, or necrotic ulcers.

The exact cause is unknown, but it is considered an immune-mediated condition, possibly triggered by infections or hypersensitivity reactions. While PLEVA involves immune system activity, it is not classified as a confirmed autoimmune disease but is suspected to have immune-mediated mechanisms.

Small, red-brown papules that may develop into vesicles, pustules, or necrotic ulcers, lesions appearing in crops, itching or burning sensation in some cases, widespread distribution primarily on the trunk and proximal extremities, potential post-inflammatory hyperpigmentation, hypopigmentation, or scarring.

Pityriasis lichenoides chronica (PLC), lymphomatoid papulosis, viral infections (e.g., Epstein-Barr virus, varicella-zoster virus, HIV), bacterial infections (e.g., Group A streptococcus, Toxoplasma gondii), rare progression to cutaneous T-cell lymphoma.

PLEVA can affect individuals of any age but is more common in children and young adults, with a slight male predominance. The exact prevalence is unknown due to its rarity. Potential risk factors include recent infections, immune system dysregulation, and genetic predisposition.

• Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA) (2023)
• Pityriasis Lichenoides Following SARS-CoV-2 Infection/Vaccination (2023)
• Immunophenotyping and viral studies in pityriasis lichenoides et varioliformis acuta lesions (2024)
• Pityriasis lichenoides: assessment of 41 pediatric patients (2024)