급성두창태선양비강진 환자 39명의 임상 및 병리조직학적 결과에 관한 고찰

Abstract

Background: Pityriasis lichenoides et varioliformis acuta (PLEVA), a type of pityriasis lichenoides disease, is an uncommon inflammatory cutaneous disorder of unknown etiology. PLEVA presents as acute inflammatory papules that progressively develop into erosions or necrotic crusts all over the body.

Objective: This study aimed to analyze the clinicopathological findings of 39 patients with PLEVA at a tertiary referral center in Seoul, Korea.

Methods: This was a retrospective and single-center study with a longitudinal section. Medical records and telephone interviews were used to review the data of patients diagnosed with PLEVA between 2006 and 2019.

Results: This study included 39 patients with PLEVA (male: female=2.9:1). The mean age of onset was 30.1 years, and 20/39 (51%) patients were children or young adults (＜30 years old). The mean duration of the disease course was 8.9 months. The disease was associated with a wide spectrum of clinical features ranging from erythematous inflammatory papules to diffusely progressing ulcers or crusted lesions. Treatment modalities involved topical corticosteroids (67%), low-dose systemic corticosteroids (44%), systemic antibiotics (33%), narrow-band ultraviolet B treatment (18%), and observation (13%). Except in one case, there were no reports of intolerable comorbidity or severe side effects within the follow-up period.

Conclusion: PLEVA showed a male predominance and primarily occurred during childhood or young adulthood in our study cohort. PLEVA could be associated with cutaneous T-cell lymphoma. Various treatment modalities may be used according to the clinical severity, and most patients exhibited a good response to treatment.