Nazzaro V, Blanchet-Bardon C, Mimoz C, Revuz J, Puissant A
Clinique des Maladies Cutanées, Hôpital Saint-Louis, Paris, France.
Arch Dermatol. 1988 Apr;124(4):533-9.
Four siblings affected by Papillon-Lefèvre syndrome (PLS) ranged in age from 2 to 11 years. The parents were unaffected and parental consanguinity was present. The 2-year-old girl showed the early manifestations of PLS; that is, slight gingival swelling and erythema occurring simultaneously with minimal scaling of palms and soles. The other siblings, aged 5, 8, and 11 years, showed severe periodontopathy with tooth loss and marked palmoplantar keratoderma with a centripetal extension of the keratoses to the limbs and trunk. These three older siblings were treated with acitretin (Ro 10-1670), the free acid of etretinate, with complete clearing of the skin and healing of gingival pockets. Treatment was given for 16 months; teeth that erupted during therapy were free of periodontopathy and remained firmly anchored to the alveolar bone. In two of the children ultrastructural examination of involved skin was performed before and during acitretin treatment. Before treatment a large number of lipidlike vacuoles were found in corneocytes and in granulocytes; tonofilaments were reduced in number, and keratohyaline granules frequently showed a rectangular or globular shape. During treatment with acitretin these abnormalities diminished markedly. Thus, etretin is effective in treating PLS and, if treatment is started at an early age, should allow patients with PLS to have normal adult dentition.
四名患有掌跖角化牙周破坏综合征（PLS）的兄弟姐妹年龄在2岁至11岁之间。父母未受影响，存在父母近亲结婚情况。两岁女童表现出PLS的早期症状，即牙龈轻度肿胀和红斑，同时伴有手掌和脚底轻微脱屑。其他三名兄弟姐妹，年龄分别为5岁、8岁和11岁，表现出严重的牙周病伴牙齿脱落，以及明显的掌跖角化病，角质化向四肢和躯干呈向心性扩展。这三名年长的兄弟姐妹接受了阿维A（Ro 10 - 1670）治疗，阿维A是维甲酸的游离酸，皮肤完全清除，牙龈袋愈合。治疗持续了16个月；治疗期间萌出的牙齿没有牙周病，并且牢固地附着在牙槽骨上。对其中两名儿童在阿维A治疗前和治疗期间进行了受累皮肤的超微结构检查。治疗前，在角质形成细胞和粒细胞中发现大量脂样空泡；张力丝数量减少，透明角质颗粒常呈长方形或球形。在阿维A治疗期间，这些异常明显减轻。因此，阿维A对治疗PLS有效，如果在早期开始治疗，应该能使PLS患者拥有正常的恒牙列。
