Fardos Mohammad I, Nikakis Jacqueline, Anagnostis Sophia, Moon Summer
Dermatology, HCA Florida Largo Hospital, Largo, USA.
General Medicine, Peconic Bay Medical Center/Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Riverhead, USA.
Cureus. 2024 Jul 19;16(7):e64908. doi: 10.7759/cureus.64908. eCollection 2024 Jul.
Lichen sclerosus (LS) is a chronic inflammatory disorder primarily affecting the anogenital region, with a higher prevalence in females and often linked to autoimmunity. This association is not clearly elucidated in males, with LS commonly presenting in uncircumcised males. The most affected areas include the glans penis, prepuce, and coronal sulcus. In this report, we present an 11-year case of treatment-resistant LS in a male patient with an extensive history of autoimmune disorders, manifesting in the intergluteal cleft as a hypertrophic plaque, a rare location. The patient had a complex autoimmune history, including porphyria cutanea tarda, discoid lupus, and Sjogren's syndrome. Histopathological analysis confirmed a diagnosis of erosive LS. Despite numerous treatments, including intralesional corticosteroids and various topicals, the lesion persisted. This case highlights the challenges in managing LS, particularly in uncommon sites and in patients with extensive autoimmune backgrounds. Treatment goals for LS focus on symptom relief, cosmetic improvement, and disease prevention. Although topical corticosteroids are commonly used, systemic options like hydroxychloroquine may be beneficial in resistant cases, although clear guidelines are lacking. Our case underscores the importance of a multidisciplinary approach in addressing LS and its associated autoimmune conditions.
硬化性苔藓(LS)是一种主要累及肛门生殖器区域的慢性炎症性疾病,女性患病率较高,且常与自身免疫有关。这种关联在男性中尚未明确阐明,LS常见于未行包皮环切术的男性。最常受累的部位包括阴茎头、包皮和冠状沟。在本报告中,我们介绍了一名患有11年难治性LS的男性患者,该患者有广泛的自身免疫性疾病病史,病变表现为臀间裂处的肥厚性斑块,这是一个罕见的部位。该患者有复杂的自身免疫病史,包括迟发性皮肤卟啉病、盘状红斑狼疮和干燥综合征。组织病理学分析确诊为糜烂性LS。尽管进行了多种治疗,包括皮损内注射皮质类固醇和各种外用药物,但病变仍持续存在。该病例突出了管理LS的挑战,特别是在罕见部位以及有广泛自身免疫背景的患者中。LS的治疗目标侧重于缓解症状、改善外观和预防疾病。尽管常用外用皮质类固醇,但对于难治性病例,羟氯喹等全身性药物可能有益,尽管缺乏明确的指南。我们的病例强调了多学科方法在处理LS及其相关自身免疫性疾病中的重要性。