Matard Bruno, Bourrat Emmanuelle, Cavalié Marine, Chiaverini Christine, Reygagne Pascal
Sabouraud Centre for Hair Diseases, Hôpital Saint-Louis, Paris, France.
Department of Dermatology, Expert Centre for Epidermolysis Bullosa (MAGEC), Hôpital Saint-Louis, Paris, France.
Br J Dermatol. 2022 Dec;187(6):1026-1028. doi: 10.1111/bjd.21796. Epub 2022 Sep 6.
This work reports 30 cases of folliculitis decalvans (FD) in patients with dystrophic epidermolysis bullosa (DEB) among a cohort of 125 DEB patients seen between 2010 and 2021 in 2 French expert centers for the management of inherited epidermolysis bullosa. Such an association between two rare diseases cannot be fortuitous and implies a physiopathological link that we discuss in this paper. This association is a new significant fact to add to the reflexion on FD causes, suggesting that skin abnormality of DEB could act as a factor of a specific skin barrier alteration which could favor FD. Scarring alopecia with tufted folliculitis and pustules on inflamed skin at the vertex of a woman with dominant dystrophic epidermolysis bullosa.
这项研究报告了在2010年至2021年间,于法国2个遗传性大疱性表皮松解症管理专家中心就诊的125例营养不良性大疱性表皮松解症(DEB)患者中,有30例发生了脱发性毛囊炎(FD)。两种罕见疾病之间的这种关联并非偶然,意味着存在一种我们在本文中讨论的病理生理联系。这种关联是在对FD病因的思考中新增的一个重要事实,表明DEB的皮肤异常可能作为一种特定皮肤屏障改变的因素,从而促成FD。一名患有显性营养不良性大疱性表皮松解症的女性,其头顶发炎皮肤上出现簇状毛囊炎和脓疱的瘢痕性脱发。