Pyostomatitis Vegetans And Orofacial Granulomatosis: A Case Report And Review Of The Literature

Thomas P. Sollecito; Eric T. Stoopler; Shrikanth Rangarajan; Andre Pinto

摘要

Pyostomatitis vegetans (PV) is a rare, chronic inflammatory condition characterized by pustules, erosions and vegetations of the skin. Oral lesions of PV appear similar to dermatologic lesions and may present on the gingiva, as well as the labial or buccal mucosa. PV has a strong association with gastrointestinal disease and is considered a marker for inflammatory bowel disease. We describe a case of a 9 year old female who demonstrated PV lesions on the oral mucosa accompanied by orofacial granulomatosis. Introduction Pyostomatitis vegetans (PV) is a rare, benign chronic disease characterized by miliary pustules, erosions and vegetating dermatosis of the skin and oral mucosa 1, 2, 3. These asymmetrical lesions typically affect the axillary folds and groin, and to a lesser extent involve the face and the scalp. 2, 4, 5 Oral mucosal lesions can involve the labial attached gingiva, as well as the labial and buccal mucosa. Oral lesions are distinct and appear as multiple pustules with an erythematous base that coalesce and undergo necrosis to form a typical “snail tracks” appearance.5, 6 PV has a strong association with gastrointestinal disease and is considered a marker for inflammatory bowel disease (IBD). 3, 5,6,7,8,9 In 1898, Hallopeau reported on five patients that presented with unusual pustular dermatosis the he called pyodermite vegetante. 8 Oral lesions that manifested throughout the mucosa were reported in two of these cases. McCarthy coined the term pyostomatitis vegetans, which he believed was a variant of the pyodermite vegetante, when he described three cases that displayed comparable oral lesions on initial presentation. 10 Since McCarthy's report in 1949, approximately 41 cases have been published in the literature.The association of PV to other mucosal diseases which may present with intraepithelial abscesses and acantholysis including pyodermite vegetante (PDV), pemphigus vegetans and pemphigus vulgaris, is controversial. 10, 11 Pemphigus vulgaris consists of poorly healing, non-vegetating, and rapidly rupturing bullae, that progress to ulcers on the oral mucosa and skin. Unlike PV, pemphigus vulgaris is a potentially fatal, well characterized autoimmune mucocutaneous disease with the presence of auto antibodies and where diagnosis is confirmed by both positive direct and indirect immunofluorescence. Pemphigus vegetans, considered a variant of pemphigus vulgaris, 12 is characterized by a less severe course and the two are differentiated by the presence of vegetations along the ruptured bullae margins. It has also been associated with intraepithelial abscesses. The occurrence of bullae, acantholysis, and a possible fatal outcome 12 depict common features evident in both conditions and distinguish them from PV. Clinically, PDV and PV feature localized pustules but lack rupturing bullae. Histologically, peripheral eosinophilia and miliary abscesses are distinct features of PDV and PV, although pemphigus vegetans may also display eosinophilia. Immunofluorescence studies have also been key in distinguishing between PV and pemphigus vulgaris. Both pemphigus vegetans and pemphigus vulgaris reveal strong intercellular deposits of IgG and C3 with direct immunofluorescence staining, and show positive circulating antibodies with indirect immunofluorescence .4 However, the pattern of immunofluorescence staining in PDV and PV ranges from negative to weak for both direct and indirect techniques. The atypical immunofluorescence in some PV cases may be the result of a secondary response to epithelial damage as opposed to pemphigus, where antibodies represent the primary response .11,13 Moreover, a weak response to immunofluorescence from a diagnostic viewpoint does not exclude a diagnosis of PDV or PV especially when typical and histologic findings are present. With a given diagnosis of PV, other reactive cutaneous and oral lesions may be evident in association with IBD such as erythema nodosum, pyoderma gangrenosum and aphthou

机译：植物性脓疱病（PV）是一种罕见的慢性炎症性疾病，其特征是皮肤上的脓疱，糜烂和植被。 PV的口腔病变看起来与皮肤科病变相似，并且可能出现在牙龈以及唇或颊粘膜上。 PV与胃肠道疾病密切相关，被认为是炎症性肠病的标志物。我们描述了一个9岁女性的案例，该女性在口腔粘膜上显示出PV病变并伴有口面肉芽肿。前言植物性脓疱病（PV）是一种罕见的良性慢性疾病，其特征是粟粒性脓疱，皮肤和口腔粘膜1、2、3糜烂和植物性皮肤病。这些不对称病变通常会影响腋窝皱和腹股沟，并且程度较小涉及面部和头皮。 2，4，5口腔粘膜病变可累及附着在唇上的龈以及唇和颊粘膜。口腔病变明显，表现为多个脓疱，具有红斑性基底，并合并坏死形成典型的“蜗牛痕迹”。5，6 PV与胃肠道疾病密切相关，被认为是炎症性肠病（IBD）的标志物）。 3、5、6、7、8、9 1898年，Hallopeau报告了5名患有异常脓疱性皮肤病的患者，他将其称为pyodermite vegetante。其中有两个病例报告了在整个粘膜上表现出的8个口腔损伤。麦卡锡（McCarthy）创造了术语“植物性脓性湿疹”，他认为这是一种“软锰矿”的变体，他描述了三例在初次就诊时表现出可比的口腔病变的病例。 10自1949年麦卡锡（McCarthy）报告以来，已有约41例病例发表.PV与其他可能伴有上皮内脓肿和棘层松解症的黏膜疾病的关系颇有争议，其中包括软性黄腐病（PDV），天疱疮性天疱疮和寻常性天疱疮。 10，11寻常型天疱疮由愈合不良，无植物和快速破裂的大疱组成，大疱进展为口腔粘膜和皮肤上的溃疡。与PV不同，寻常性天疱疮是一种潜在的致命，特征明确的自身免疫性粘膜皮肤疾病，存在自身抗体，并且通过阳性直接和间接免疫荧光都可以确诊。蔬菜天疱疮，被认为是寻常性天疱疮的一种变种，其特点是病程较轻，其特征是沿破裂的大疱边缘存在植被，从而使二者区别开来。它也与上皮内脓肿有关。大疱，棘皮松解症和可能的致命结果的发生12描绘了在两种情况下均显而易见的共同特征，并将其与PV区别开来。临床上，PDV和PV具有局部脓疱，但缺乏破裂的大疱。从组织学上来说，外周血嗜酸性粒细胞增多和粟粒性脓肿是PDV和PV的明显特征，尽管天疱疮素食主义者也可能表现出嗜酸性粒细胞增多。免疫荧光研究也是区分PV和寻常型天疱疮的关键。天然天疱疮和寻常型天疱疮都显示出强烈的IgG和C3细胞间沉积，并带有直接免疫荧光染色，并显示带有间接免疫荧光的循环阳性抗体。4但是，PDV和PV中直接和间接的免疫荧光染色模式从负到弱技术。在某些PV病例中，非典型免疫荧光可能是对天疱疮损害的继发性反应而非天疱疮的结果，其中天疱疮以抗体为主要反应。11,13此外，从诊断的角度来看，对免疫荧光的弱反应并不排除诊断为PDV或PV，尤其是在存在典型的组织学表现时。在诊断为PV的情况下，与IBD相关的其他皮肤和口腔反应性病变可能很明显，例如结节性红斑，坏疽性脓皮病和口疮

Pyostomatitis Vegetans And Orofacial Granulomatosis: A Case Report And Review Of The Literature

摘要

著录项

相似文献

相关主题

期刊订阅