Autoimmune Bullous Diseases Research Center, Razi Dermatology Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Australas J Dermatol. 2019 May;60(2):e105-e108. doi: 10.1111/ajd.12923. Epub 2018 Sep 14.
BACKGROUND/OBJECTIVES: The aim of this study was to describe a previously unreported association of oral pemphigus vulgaris with short-lived blood-filled painless blisters resembling angina bullosa haemorrhagica (ABH).
A cross-sectional study of consecutive patients with Pemphigus vulgaris. All patients were examined for the presence of ABH-like lesions, and demographic, clinical and histopathological data were collected. Histopathological examination was performed when feasible.
A total of 318 with pemphigus vulgaris were included (63.5% female, mean age: 46 years). ABH-like lesions were present in 82 (25.8%) patients, commonly observed in the buccal mucosa (47, 57.3%) followed by the palate (15, 18.3%). All patients had normal platelet counts with no evidence of bleeding diathesis. Biopsies of the ABH-like lesions showed suprabasal clefts in four of six samples. ABH-like lesions were significantly associated with partial remission of pemphigus vulgaris (47.5%, P = 0.002) and the use of intraoral steroids (P = 0.001, odds ratio: 5.9 [95% confidence interval: 2.5-13.6]).
ABH-like lesions may represent a transient or abortive form of oral pemphigus vulgaris and tend to have a benign and self-limiting nature.
背景/目的:本研究旨在描述一种以前未报道的口腔寻常型天疱疮与短暂性无痛血性水疱(类似出血性大疱性类天疱疮)之间的关联。
对连续的寻常型天疱疮患者进行横断面研究。所有患者均检查有无类似 ABH 的病变,并收集人口统计学、临床和组织病理学数据。在可行的情况下进行组织病理学检查。
共纳入 318 例寻常型天疱疮患者(女性占 63.5%,平均年龄:46 岁)。82 例(25.8%)患者存在类似 ABH 的病变,常见于颊黏膜(47 例,57.3%),其次为腭部(15 例,18.3%)。所有患者血小板计数正常,无出血倾向。六例 ABH 样病变活检中有四例显示棘层上方裂隙。ABH 样病变与寻常型天疱疮部分缓解(47.5%,P=0.002)和口腔内类固醇的使用显著相关(P=0.001,优势比:5.9[95%置信区间:2.5-13.6])。
ABH 样病变可能代表口腔寻常型天疱疮的短暂或中止形式,倾向于具有良性和自限性。