Department of Dermatology, University of Lübeck, Lübeck, Germany; Department of Dermatology, University of Heidelberg, Medical Faculty Mannheim, Mannheim, Germany.
Department of Dermatology, University of Lübeck, Lübeck, Germany; Charité - Universitätsmedizin, corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin, Institute of Pathology, Berlin, Germany.
Acta Derm Venereol. 2023 Aug 25;103:adv11947. doi: 10.2340/actadv.v103.11947.
Mucous membrane pemphigoid is an autoimmune blistering disorder characterized by predominant involvement of surface-close epithelia and linear depositions of immunoreactants at the dermal-epithelial junction on direct immunofluorescence microscopy. A major diagnostic difficulty is the frequent need for multiple biopsies to facilitate the diagnosis. Although oesophageal involvement is a rare, but life-threatening manifestation, the relevance of oesophageal direct immunofluorescence sampling is unclear. This retrospective monocentric study evaluated 67 non-lesional biopsies from 11 patients with mucous membrane pemphigoid and clinical symptoms suggestive of oesophageal involvement, comprising 31 samples from the oesophagus and 36 samples from other anatomical sites. Five patients (45.5%) exhibited endoscopic findings compatible with oesophageal involvement of mucous membrane pemphigoid. No correlation was identified between the presence of oesophageal lesions and direct immunofluorescence positivity in lesions from the oesophagus (p = 1.0). Oral and cutaneous samples were significantly more frequently positive by direct immunofluorescence than were oesophageal biopsies (p < 0.0001 and p = 0.0195, respectively). Oesophageal samples yielded significantly less IgG reactivity than oral and cutaneous lesions (p < 0.0001 and p = 0.0126, respectively), and less IgA antibody response than oral lesions (p = 0.0036). In conclusion, oesophageal direct immunofluorescence samples were inferior to oral and cutaneous biopsies for the diagnosis of mucous membrane pemphigoid even when oesophageal lesions compatible with mucous membrane pemphigoid were present at the time of biopsy.
黏膜类天疱疮是一种自身免疫性水疱性疾病，其特征是主要累及表面接近的上皮和在直接免疫荧光显微镜下真皮-上皮交界处的免疫反应物线性沉积。主要的诊断困难是频繁需要多次活检以促进诊断。虽然食管受累是一种罕见但危及生命的表现，但食管直接免疫荧光采样的相关性尚不清楚。这项回顾性单中心研究评估了 11 名黏膜类天疱疮患者的 67 例非病变活检，这些患者有临床症状提示食管受累，包括 31 例食管样本和 36 例其他解剖部位样本。5 名患者（45.5%）表现出与黏膜类天疱疮的食管受累相符的内镜发现。在食管病变中，食管病变的存在与直接免疫荧光阳性之间未发现相关性（p=1.0）。口腔和皮肤样本的直接免疫荧光阳性率明显高于食管活检（p<0.0001 和 p=0.0195）。食管样本的 IgG 反应性明显低于口腔和皮肤病变（p<0.0001 和 p=0.0126），而 IgA 抗体反应性明显低于口腔病变（p=0.0036）。总之，即使在活检时存在与黏膜类天疱疮相符的食管病变，食管直接免疫荧光采样也不如口腔和皮肤活检对黏膜类天疱疮的诊断有价值。
