Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon.
J Am Acad Dermatol. 2010 Dec;63(6):1036-41. doi: 10.1016/j.jaad.2009.08.067. Epub 2010 Oct 8.
Pachydermoperiostosis (PDP) is a rare hereditary disorder characterized by digital clubbing, periostosis, and pachydermia. Pachydermia results in leonine facies, a major cause of cosmetic and functional morbidity in these patients. Its treatment is usually surgical. So far, no medical treatment has been suggested to alleviate this morbidity.
We sought to assess the role of botulinum toxin type A (BTX-A) in improving the cosmetic appearance of pachydermia in patients with PDP.
Three patients with PDP were treated with BTX-A for their leonine facies. A total of 70 to 80 U were used to treat the upper third of the face. Photographs were taken at baseline and at 2 and 6 weeks after the injections. The patients were followed up periodically for at least 6 months. Wrinkle severity was assessed at relaxation using the 4-point facial wrinkle scale at baseline, week 6, and month 6. In addition, a subjective assessment of the improvement of the extent and depth of the facial rhytides/furrows over the upper third of the face was performed by the same investigator at week 6 and month 6.
Using the subjective assessment of the improvement of wrinkles, all 3 patients exhibited a fair to excellent response at week 6 that started manifesting 1 week after the BTX-A treatment. All patients demonstrated a residual effect 6 months after the treatment. One patient exhibited a mild exacerbation of his ptosis.
Major limitations were the small number of patients and the administration of BTX-A injections and assessment of their response by a single unblinded physician.
BTX-A is a simple procedure that may be of value in temporarily improving the cosmetic appearance of pachydermia in patients with PDP.
厚皮性骨膜病（Pachydermoperiostosis，PDP）是一种罕见的遗传性疾病，其特征为杵状指（趾）、骨膜增生和皮肤变厚。皮肤变厚导致狮面，这是这些患者出现美容和功能障碍的主要原因。其治疗通常为手术。到目前为止，还没有提出任何药物治疗来减轻这种发病率。
我们旨在评估肉毒毒素 A 型（Botulinum Toxin Type A，BTX-A）在改善 PDP 患者厚皮病的美容外观方面的作用。
对 3 例 PDP 患者的狮面采用 BTX-A 进行治疗。总共使用 70-80U 来治疗面部的上三分之一。在基线、注射后 2 周和 6 周拍摄照片。对患者进行定期随访，至少 6 个月。在基线、第 6 周和第 6 个月时使用 4 分面部皱纹量表评估松弛时皱纹的严重程度。此外，在第 6 周和第 6 个月，同一位研究者对治疗上三分之一面部皱纹/纹路的程度和深度的改善进行了主观评估。
使用皱纹改善的主观评估，所有 3 例患者在 BTX-A 治疗后 1 周开始出现，第 6 周时均表现出良好至极好的反应。所有患者在治疗后 6 个月仍有残余效果。1 例患者出现轻度上睑下垂加重。
主要局限性是患者数量少，以及由单一非盲医生进行 BTX-A 注射和对其反应的评估。
BTX-A 是一种简单的程序，可能对改善 PDP 患者厚皮病的美容外观有一定价值。
