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A Clinicopathological Study Of 22 Cases Of Pemphigus

机译:天疱疮22例临床病理研究

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Background : The term pemphigus refers to a group of autoimmune blistering diseases of the skin and the mucous membranes . Pemphigus affects 0.1- 0.5 patients per 1,00,000 population per year . Aim: To evaluate the clinical findings in association with histopathological findings, to study the histopathological findings in various vesiculobullous lesions of skin and to confirm with immunofluorescence wherever possible. Materials And Methods: Histopathological evaluation of biopsies received from the department of Dermatology was done in the Department of Pathology, JSS hospital, Mysore, from August 2006 to July 2008. Results: A total number of 648 skin biopsies were received, out of which 22 cases were of the pemphigus group of diseases. Pemphigus vulgaris accounted for 81 %( 18) of cases and pemphigus vegetans accounted for 9.01 %( 2) of cases. The less common types were pemphigus erythematosus and IgA pemphigus constituting 4.1 %( 1) of the cases. Pemphigus vulgaris formed the most common variant. The male to female ratio was 1.35:1. The age incidence was between 21-70 years .The trunk and extremities were the frequently involved sites and had classical flaccid bullae in 90.9% of cases. Oral mucosa was involved in 66% of the cases of pemphigus vulgaris. Apart from the common histopathological changes in pemphigus vulgaris, we also observed: 1.Spongiosis of adjacent epidermis (11 cases) 2. Acantholysis in adnexa (4cases) 3. Hair shaft and sebaceous gland inside bulla (biopsy artifact)(3cases). Both the cases of pemphigus vegetans showed hyperkeratosis, papillomatosis and acanthosis, along with suprabasal bulla containing inflammatory infiltrate. Conclusion; Histopathological features were conclusive in most of the cases of primary vesiculobullous lesions of the skin. The study also revealed additional histopathological findings which are not encountered normally. Analysis of the subtle light microscopic features apart from the classical diagnostic features assisted in the diagnosis of difficult cases. The immunofluorescence study done, helped in confirming the diagnosis where histopathology and clinical features alone were inconclusive.
机译:背景:天疱疮是一组皮肤和粘膜的自身免疫性水疱病。天疱疮每年影响每1000万人口中0.1- 0.5名患者。目的:评估与组织病理学发现相关的临床发现,研究皮肤各种囊泡性病变中的组织病理学发现,并在可能的情况下进行免疫荧光确认。材料与方法:2006年8月至2008年7月,在迈索尔JSS医院病理科对皮肤科进行的活检进行了组织病理学评估。结果:总共接受了648例皮肤活检,其中22例病例属于天疱疮组。寻常型天疱疮占病例的81%(18),而天疱疮的素食者占病例的9.01%(2)。较不常见的类型是红斑痣,IgA天疱疮,占病例的4.1%(1)。寻常型天疱疮是最常见的变种。男女比例为1.35:1。年龄发生在21-70岁之间。躯干和四肢是最常见的部位,在90.9%的病例中患有典型的松弛性大疱。 66%的寻常型天疱疮病例涉及口腔粘膜。除寻常性天疱疮的常见组织病理学改变外,我们还观察到:1.相邻表皮的椎管炎(11例)2.附件棘皮松解术(4例)3.大疱内的毛干和皮脂腺(活检伪影)(3例)。天疱疮性蔬菜的两例均表现为角化过度,乳头状瘤病和棘皮症,以及含有炎性浸润的基底上大疱。结论;在大多数原发性囊泡性皮肤病变病例中,组织病理学特征是决定性的。该研究还揭示了其他正常情况下不会遇到的组织病理学发现。除了经典的诊断功能外,对微光镜特征的分析有助于诊断疑难病例。进行的免疫荧光研究有助于确定仅组织病理学和临床特征尚无定论的诊断。

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