探索寻常型天疱疮的临床谱系和个体化药物治疗:病例系列

Evangeline Gladwin, Priyanshi Shah, Himani Shah, Krunal Tralsawala
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摘要

丘疹性荨麻疹(Pemphigus Vulgaris,PV)是一种罕见的、慢性的、危及生命的免疫致病性疾病,其特征是松弛的、易破裂的上皮内大疱,主要出现在皮肤和粘膜上。为了使这种可能致命的疾病的治疗方案成为主要焦点,我们观察了五名寻常型天疱疮患者。所有患者都有口腔病变或溃疡,导致吞咽困难。其中一名患者有家族病史,其他患者则没有任何家族病史。五名患者在入院前都有不同的处理方式。他们都患有小红细胞性贫血和低白蛋白血症。1 号和 3 号患者白细胞增多,而 3 号患者血小板成团,伴有高钴胺血症,尿液分析中出现酮体。去甲斑蝥素 I 和去甲斑蝥素 III 抗体以及冲孔活检结果证实了这一诊断。所有患者在住院期间都接受了全身皮质类固醇治疗,但该病例系列观察到的剂量差异强调了药物治疗的个体化。一些患者出现了心理障碍,一些患者出现了全身性头痛或心功能损害,而其他患者则出现了眼部受累。医护人员在提供个体化药物治疗的同时,应进行密切监测,以防止出现任何全身性并发症。
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EXPLORING THE CLINICAL SPECTRUM AND INDIVIDUALIZED DRUG THERAPY IN PEMPHIGUS VULGAR IS: A CASE SERIES
Pemphigus Vulgaris (PV) is a rare, chronic, life-threatening immunopathogenic disease that is characterized by flaccid, easily ruptured intraepithelial bullae, mostly found on the skin and mucous membranes. Attempting to keep the treatment plan of this potentially deadly disorder in primary focus, we have observed five patients with Pemphigus vulgaris. All patients have been presented with oral lesions or ulcers resulting in difficulty in swallowing. Patient one had a family history of the disease, while the other patients did not present any family history. All five patients had diverse ways of dealing with the disease before getting hospitalized. Microcytic anaemia and hypoalbuminemia have been found in all of them. Patients 1 and 3 had leucocytosis, while patient three had platelets in clumps with Hypercobalaminemia and the presence of ketone bodies in urine analysis. The Desmoglein I and Desmoglein III Antibody along with Punch biopsy results, confirmed the diagnosis. All patients were given systemic corticosteroids on hospitalization, but the case series observed variations in the dosages emphasizing individualized drug therapy. Some developed psychological impairment, some had generalized headaches or cardiac impairment, while ophthalmic involvement was seen in the other patients. Healthcare providers should do close monitoring while providing individualized drug therapy to prevent any systemic involvement and further complications.
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