Sandesh Shah

and 2 more

IntroductionPityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha-Habermann disease, is a rare T-cell lymphoproliferative disorder that can occur at any age but most commonly affects children and young adults, with a slight male preponderance.1 The pathogenesis of PLEVA is thought to involve two mechanisms: a T-cell dyscrasia and an aberrant immune response to viral, bacterial, or protozoal infections.2PLEVA is marked by the sudden appearance of asymptomatic to multiple small erythematous papules that rapidly progress into polymorphic lesions with varying forms, including vesicles, pustules, hemorrhagic crusted papules, and shallow ulcers. These lesions typically resolve within weeks to months but can occasionally leave behind long-lasting complications such as hyperpigmented or hypopigmented, varioliform scars. The lesions primarily affect the anterior trunk, flexural areas, and proximal extremities, though they can also occur in other parts of the body, sparing the mucosa.1,3PLEVA typically has a self-limiting course, with lesions resolving spontaneously over time. However, in certain cases, the condition may persist and follow a more recalcitrant trajectory, characterized by recurring flare-ups and varying lengths of remission between episodes.3To our knowledge, this is the first documented case of PLEVA presenting with significant facial and mucosal involvement in a 15-year-old male from Nepal. This case highlights the atypical nature of the condition and underscores the rarity of such presentations.