Király Zsófia, Kovács Anikó, Medvecz Márta, Róbert Lili, Bokor Laura, Kuroli Enikő, Szepesi Ágota, Marschalkó Márta, Hidvégi Bernadett
1 Semmelweis Egyetem, Általános Orvostudományi Kar, Bőr-, Nemikórtani és Bőronkológiai Klinika Budapest, Mária u. 41., 1085 Magyarország.
2 Semmelweis Egyetem, Általános Orvostudományi Kar, Patológiai és Kísérleti Rákkutató Intézet Budapest Magyarország.
Orv Hetil. 2023 Feb 5;164(5):172-178. doi: 10.1556/650.2023.32692.
Lupus erythematous panniculitis (LEP) is a rare type of chronic cutaneous lupus erythematous. Clinical characteristics are tender, subcutaneous nodules, erythematous plaques. Disfigurement of face and body might develop which affects the patient's quality of life. LEP can be the first sign of systemic lupus erythematous (SLE).
Our aim was to review the clinicopathological characteristics and the course of LEP through our own patients.
We retrospectively analyzed the clinical records of 17 LEP patients at Semmelweis University's Department of Dermatology, Venerology and Dermatooncology between 2000 and 2022.
The male : female ratio was 1 : 16, average age was 37.8 years. Lesion localisations were proximal lower (8/17) and upper extremities (7/17), face (4/17), breast (3/17), chest (2/17), buttocks (2/17), back (1/17) and distal lower extremity (1/17). Lesion morphologies were nodules (11/17), plaques (7/17), lipoatrophy (4/17), ulceration (3/17), calcification (1/17). Discoid changes covered in 6 cases. In 10 cases, systemic symptoms were observed (arthritis (4/17), haematological (5/17), renal (2/17), anti-phospholipid syndrome (2/17). 7 patients fulfilled the EULAR/ACR criteria for SLE. Histology showed mixed type panniculitis in 8, lobular in 3 cases. Average time until diagnosis was 24.3 months. Among all our SLE patients, skin symptoms regressed following systemic immunosuppressive treatment. LEP patients with only skin manifestation were often resistant for the therapy of cutaneous lupus erythematous.
The diagnosis of LEP often takes months or years. Wider knowledge of LEP would shorten the time to diagnosis, preventing disfigurement and possible damage of internal organs. Based on our observations, LEP without SLE might be treated with early immunosuppression. Orv Hetil. 2023; 164(5): 172-178.
狼疮性脂膜炎（LEP）是一种罕见的慢性皮肤型红斑狼疮。临床特征为压痛性皮下结节、红斑性斑块。面部和身体可能出现毁容，影响患者生活质量。LEP可能是系统性红斑狼疮（SLE）的首发症状。
我们的目的是通过自身患者回顾LEP的临床病理特征及病程。
我们回顾性分析了2000年至2022年间塞梅尔维斯大学皮肤科、性病与皮肤肿瘤学系17例LEP患者的临床记录。
男女比例为1∶16，平均年龄为37.8岁。皮损部位为下肢近端（8/17）、上肢近端（7/17）、面部（4/17）、乳房（3/17）、胸部（2/17）、臀部（2/17）、背部（1/17）和下肢远端（1/17）。皮损形态为结节（11/17）、斑块（7/17）、脂肪萎缩（4/17）、溃疡（3/17）、钙化（1/17）。6例出现盘状改变。10例观察到全身症状（关节炎（4/17）、血液系统症状（5/17）、肾脏症状（2/17）、抗磷脂综合征（2/17））。7例患者符合欧洲抗风湿病联盟/美国风湿病学会（EULAR/ACR）SLE标准。组织学显示8例为混合型脂膜炎，3例为小叶型。确诊平均时间为24.3个月。在我们所有的SLE患者中，全身免疫抑制治疗后皮肤症状消退。仅表现为皮肤症状的LEP患者通常对皮肤型红斑狼疮治疗耐药。
LEP的诊断通常需要数月或数年。对LEP有更广泛的认识将缩短诊断时间，防止毁容和可能的内脏损害。根据我们的观察，无SLE的LEP可能早期进行免疫抑制治疗。《匈牙利医学周报》。2023年；164（5）：172 - 178。
