Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.
Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.
Clin Dermatol. 2021 Mar-Apr;39(2):248-255. doi: 10.1016/j.clindermatol.2020.10.017. Epub 2020 Oct 17.
Papuloerythroderma of Ofuji (PEO) is a rare skin condition first described in 1984 and characterized by diffuse erythroderma composed of papules coalescing into plaques with sparing of skin folds, known as the deck-chair sign. The disease is almost exclusively seen in the elderly and affects men more frequently than women. Common laboratory findings include peripheral and tissue eosinophilia, elevated levels of immunoglobulin E, and lymphopenia. The diagnosis entails exclusion of potentially causative pathologies, including drug intake, atopy, malignancy, and infection. These factors have frequently been found in association with PEO, but their role in the etiopathogenesis of the disease is poorly understood. A dysregulated immune system, with particular involvement of T-helper (Th)2 and Th22 cells, seems to be important in the development of PEO. Controversy exists as to whether PEO exists as an independent entity or as a clinical pattern of a variety of distinct conditions. Treatment necessitates first addressing any coexisting circumstances that may have a causal relationship with PEO. In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.
奥菲古氏斑状红皮病(PEO)是一种罕见的皮肤疾病,于 1984 年首次描述,其特征为弥漫性红斑,由融合成斑块的丘疹组成,褶皱处皮肤保留,即“躺椅征”。该病几乎仅见于老年人,且男性发病率高于女性。常见的实验室发现包括外周和组织嗜酸性粒细胞增多、免疫球蛋白 E 水平升高和淋巴细胞减少。诊断需要排除可能的病因,包括药物摄入、特应性、恶性肿瘤和感染。这些因素常与 PEO 相关,但它们在疾病发病机制中的作用尚不清楚。免疫系统失调,特别是辅助性 T 细胞(Th)2 和 Th22 细胞的参与,似乎在 PEO 的发展中很重要。目前尚存在争议,即 PEO 是否作为一种独立实体存在,还是作为各种不同疾病的临床模式存在。治疗需要首先解决任何可能与 PEO 有因果关系的共存情况。在特发性病例中,已使用局部和口服皮质类固醇、紫外线疗法和免疫抑制/免疫调节疗法,但结果不一。需要进一步的研究来深入了解疾病过程,并为诊断评估和治疗制定指南。