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伴有多处皮肤溃疡的深部红斑狼疮:1例罕见病例

Lupus Erythematosus Profundus with Multiple Overlying Cutaneous Ulcerations: A Rare Case.

作者信息

Sutedja Endang, Widjaya Muhamad Radyn Haryadi, Dharmadji Hartati Purbo, Suwarsa Oki, Pangastuti Miranti, Usman Hermin Aminah, Firdaus Chaerani Pratiwi

机构信息

Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, West Java, Indonesia.

Department of Anatomical Pathology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, West Java, Indonesia.

出版信息

Clin Cosmet Investig Dermatol. 2023 Sep 28;16:2721-2726. doi: 10.2147/CCID.S430068. eCollection 2023.

Abstract

Lupus erythematosus profundus (LEP) is a rare subset of chronic cutaneous lupus erythematosus (CCLE), with a reported incidence of 1-3% in all LE cases. The most common cutaneous clinical presentation includes indurated plaques or subcutaneous nodules with an overlying normal skin. The clinical findings range from skin redness to features of CCLE, such as scaling, follicular plugging, and atrophy. Ulceration is rare and occurs in 28% of all LEP cases. We present a case report of LEP with multiple cutaneous ulcers on the right cheek and scalp accompanied by cicatricial alopecia. No other systemic manifestations were noted. Histopathological examination revealed periadipocyte, perivascular, and perivascular infiltration of lymphocytes, eosinophils, and plasma cells, supporting the diagnosis of LEP. The topical treatments given to the patient were sunscreen, 2% mupirocin cream, and wound dressing with dialkyl carbamoyl chloride (DACC). The patient was also treated systemically with oral corticosteroids and hydroxychloroquine. Clinical improvements were observed in the 3rd month of follow-up, and ulcer healing resulted in atrophic scars and fading erythematous macules. LEP is seldom associated with systemic or discoid lupus erythematosus. This occurs twice as frequently as a distinct entity does. Diagnosis accuracy plays an important role in determining the appropriate wound care, topical, and systemic treatments for LEP patients with multiple overlying cutaneous ulcerations.

摘要

深部红斑狼疮(LEP)是慢性皮肤型红斑狼疮(CCLE)的一种罕见亚型,据报道在所有红斑狼疮病例中的发病率为1%-3%。最常见的皮肤临床表现包括硬结性斑块或皮下结节,其上覆皮肤正常。临床症状范围从皮肤发红到CCLE的特征,如脱屑、毛囊堵塞和萎缩。溃疡很少见,在所有LEP病例中占28%。我们报告一例LEP病例,患者右脸颊和头皮出现多处皮肤溃疡,并伴有瘢痕性脱发。未发现其他全身表现。组织病理学检查显示淋巴细胞、嗜酸性粒细胞和浆细胞在脂肪细胞周围、血管周围浸润,支持LEP的诊断。给予患者的局部治疗包括防晒霜、2%莫匹罗星软膏以及用二烷基氨基甲酰氯(DACC)进行伤口敷料。患者还接受了口服糖皮质激素和羟氯喹的全身治疗。在随访的第3个月观察到临床改善,溃疡愈合导致萎缩性瘢痕和红斑性斑疹消退。LEP很少与系统性或盘状红斑狼疮相关。这种情况的发生频率是作为一种独立疾病的两倍。诊断准确性在为有多处皮肤溃疡的LEP患者确定合适的伤口护理、局部和全身治疗方面起着重要作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f2a/10544172/777ea1d0fccc/CCID-16-2721-g0001.jpg

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