Pityriasis lichenoides et varioliformis acuta: a disease spectrum

Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease (MHD), is a cutaneous disorder evident with crops of erythematous macules and papules, usually on the trunk and flexural areas of the extremities. Its etiology remains unknown. PLEVA is speculated to be an inflammatory reaction triggered by certain infectious agents, an inflammatory response secondary to T-cell dyscrasia, or an immune complex-mediated hypersensitivity. Histologic examination of a skin biopsy specimen is the standard for the identification of PLEVA, but definitive diagnosis may be difficult. Apart from the febrile ulcerative variant, which may be fatal, PLEVA tends to be self-limited in its course. Treatment is targeted mainly at the symptomatic relief of pruritus.