Chowdhary Mudit, Davila Ulysses, Cohen David J
Division of Dermatology, Department of Internal Medicine, Mercer University School of Medicine, Macon, Ga., USA.
Case Rep Dermatol. 2015 Mar 17;7(1):46-50. doi: 10.1159/000381011. eCollection 2015 Jan-Apr.
Pityriasis rubra pilaris (PRP) is an exceptionally rare, chronic inflammatory dermatosis of unknown etiology. Patients classically present with small, follicular keratosis and salmon-colored plaques that begin at the head and neck and slowly progress to widespread erythroderma including the palms and soles. It is difficult to distinguish PRP from other inflammatory dermatoses; however, features that help aid in the diagnosis include 'islands' of spared skin, orangish hue and typical findings on biopsy. There are no specific guidelines on therapy and treatment options include corticosteroids, vitamin D analogs, retinoids, methotrexate, cyclosporine, azathioprine and tumor necrosis factor alpha antagonists. Unfortunately options are limited for patients when these drugs do not work. We report a case of chronic PRP, refractory to conventional treatment, successfully treated with ustekinumab monotherapy. The patient was treated with 90 mg subcutaneous ustekinumab injections and began to show improvement within only 8 weeks. Long-term control of the disease has been attained without any significant side effects. We report this case to show that ustekinumab can be used as an alternative treatment method for patients with chronic, unremitting PRP. Treatment response is remarkably rapid and the infrequent dosing leads to patient compliance and a significantly improved quality of life.
红皮病型毛发红糠疹（PRP）是一种病因不明的极为罕见的慢性炎症性皮肤病。患者通常表现为小的毛囊角化病和鲑鱼色斑块，起于头颈部，逐渐发展为包括手掌和足底在内的广泛红皮病。PRP与其他炎症性皮肤病难以区分；然而，有助于诊断的特征包括未受累皮肤的“岛屿”状、橙色色调以及活检的典型表现。目前尚无关于治疗的具体指南，治疗选择包括皮质类固醇、维生素D类似物、维甲酸、甲氨蝶呤、环孢素、硫唑嘌呤和肿瘤坏死因子α拮抗剂。不幸的是，当这些药物无效时，患者的选择有限。我们报告一例慢性PRP患者，对传统治疗无效，采用乌司奴单抗单药治疗成功。该患者接受了90mg皮下注射乌司奴单抗治疗，仅8周内就开始显示出改善。疾病得到了长期控制，且无任何明显副作用。我们报告此病例以表明乌司奴单抗可作为慢性、持续性PRP患者的替代治疗方法。治疗反应非常迅速，且给药频率低，提高了患者的依从性并显著改善了生活质量。
