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两位同胞患者,姐29岁,弟19岁。均主诉牙龈出血和吞咽困难。追查病史,患者均有Kindler综合征的典型症状,如新生儿水疱,皮肤脆弱,且对B波长紫外线过敏。口外检查:患者手、面和颈部皮肤干燥、萎缩、有异色症、下眼睑外翻、上下唇有着色斑,伴双侧口角炎。口内检查:颊、腭、舌、口底处软组织正常,但上唇沟变浅。男性患者牙龈缘肿胀,红斑、溃疡形成,牙周袋深2～3mm,轻触即严重出血,牙龈脱屑,X线片示少量骨丧失。女性患者牙龈体征如前,有迅速发展的牙周疾病,牙齿松动置Ⅲ°,X线示严重骨丧失。在组织学上,除有牙龈炎症外,部分上皮与结缔组织分离,在基底膜区有大量小泡,泡底可见周期性萎缩细胞。直接免疫荧光技术检查IgG,IgA,IgM,C_3阴性,而天疱疮,类天疱疮,扁平苔藓则为阳性。
Two fellow patients, sister 29 years old, younger brother 19 years old. Both complained of gum bleeding and swallowing difficulties. Tracing history, patients with typical symptoms of Kindler syndrome, such as neonatal blisters, skin fragile, and B-wavelength ultraviolet light allergy. Oral examination: the patient's hand, face and neck dry skin, atrophy, heterochromia, lower eyelid valgus, upper and lower lip with pigmentation, with bilateral angular cheilitis. Mouth examination: buccal, palate, tongue, soft tissue normal mouth, but shallow upper lip groove. Gingival margin of male patients with swelling, erythema, ulcers, periodontal pocket depth 2 ~ 3mm, touch that is serious bleeding, gums scaling, X-ray showed a small amount of bone loss. Female patients with gum symptoms as before, the rapid development of periodontal disease, loose teeth set Ⅲ °, X-ray showed severe bone loss. Histologically, in addition to gum inflammation, part of the epithelium and connective tissue separation, in the basement membrane area with a large number of vesicles, the end of the cyclical atrophy can be seen at the end of the cell. Direct immunofluorescence test for IgG, IgA, IgM, C_3 negative, and pemphigus, pemphigoid, lichen plank was positive.