Peuvrel L, Barbarot S, Gagey-Caron V, Tessier M-H, Cassagnau E, Stalder J-F
Service de dermatologie, CHU de Nantes, Hôtel Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
Ann Dermatol Venereol. 2008 Nov;135(11):753-6. doi: 10.1016/j.annder.2008.02.022. Epub 2008 Jul 23.
Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa.
A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. The physical examination revealed pustular and exophytic lesions of the jugal, gingival and palatine mucosa on an erythematous background, as well as some pustular and crusted lesions of the lower lip and nostrils. Histopathological analysis revealed epithelial hyperplasia and a suprabasal cleft with some signs of acantholysis and numerous neutrophils and eosinophils. Direct and indirect immunofluorescence assay was negative. There was no associated bowel disease. We concluded on a diagnosis of PPV of younger subjects. The lesions disappeared with oral corticosteroids but with steroid dependency.
PPV is a rare dermatosis associated in more than 75% of cases with inflammatory bowel disease, usually ulcerative colitis. Lesions of the oral mucosa are a constant finding and are characterised by aseptic pustules on an erythematous background. Skin lesions are pustular and more or less exophytic. To our knowledge, there have been no reports to date of intranasal lesions of PPV.
增殖性脓皮病-增殖性口炎(PPV)是一种罕见的慢性疾病,常与炎症性肠病相关。我们报告一例仅累及口腔、唇部和鼻黏膜的病例。
一名28岁男性,全身状况良好,有2年无痛性口炎病史。体格检查发现面颊、牙龈和腭黏膜有脓疱性和外生性病变,红斑为背景,下唇和鼻孔有一些脓疱性和结痂性病变。组织病理学分析显示上皮增生和基底层上裂隙,有一些棘层松解迹象以及大量中性粒细胞和嗜酸性粒细胞。直接和间接免疫荧光试验均为阴性。无相关肠道疾病。我们诊断为青年型PPV。病变经口服糖皮质激素后消失,但出现了激素依赖。
PPV是一种罕见的皮肤病,超过75%的病例与炎症性肠病相关,通常为溃疡性结肠炎。口腔黏膜病变是常见表现,特征为红斑背景上的无菌脓疱。皮肤病变为脓疱性且或多或少为外生性。据我们所知,迄今为止尚无PPV鼻内病变的报道。