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Journal of the American Academy of Dermatology
Volume 63, n° 6
pages 1036-1041 (décembre 2010)
Doi : 10.1016/j.jaad.2009.08.067
accepted : 12 August 2009
Original Articles

Treatment of pachydermoperiostosis pachydermia with botulinum toxin type A
 

Samer Ghosn, MD a, Imad Uthman, MD b, Maurice Dahdah, MD a, Abdul Ghani Kibbi, MD a, Nelly Rubeiz, MD a,
a Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon 
b Department of Internal Medicine, Division of Rheumatology, American University of Beirut Medical Center, Beirut, Lebanon 

Correspondence to: Nelly Rubeiz, MD, Department of Dermatology, American University of Beirut Medical Center, PO Box 11-0236, Riad El Solh St, Beirut, Lebanon.
Abstract
Background

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.

Objective

We sought to assess the role of botulinum toxin type A (BTX-A) in improving the cosmetic appearance of pachydermia in patients with PDP.

Methods

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.

Results

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.

Limitations

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.

Conclusion

BTX-A is a simple procedure that may be of value in temporarily improving the cosmetic appearance of pachydermia in patients with PDP.

The full text of this article is available in PDF format.

Key words : botulinum toxin type A, leonine facies, pachydermia, pachydermoperiostosis, primary hypertropic osteoarthropathy

Abbreviations used : BTX-A, FWS, PDP, PGE2



 Funding sources: None.
 Conflicts of interest: None declared.
 Reprints not available from the authors.



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