The mask of furrow: A case of a 28-year-old Filipino male with pachydermoperiostosis.
- Author:
Precious Imam
1
;
Elizabeth P. Prieto
;
Vilma Pelino
;
Milali Torres
;
Monique Lianne Lim Ang
Author Information
- Publication Type:Journal Article
- Keywords: pachydermoperiostosis; primary hypertrophic osteoarthropathy
- From: Journal of the Philippine Medical Association 2021;100(1):51-57
- CountryPhilippines
- Language:English
-
Abstract:
Pachydermoperiostosis (PDP) or primary
hypertrophic osteoarthropathy (PHO) is a rare
genetic disorder that affects mainly skin and bones.
Its main clinical features are pachyderma
(thickening of the skin), periostosis ~excessiv~ bone
formation) and clubbing of fingernails. The d1sea~e
is more common among males with a 7:1 ratio,
starts during adolescence and stabilize and cease
progression after 5 - 20 years.
OBJECTIVE:To discuss the summary of the case, new management options, and outcomes of the management options.
CASE SUMMARY (METHODS):A 28-year-old male presents with a 9-year history of wrinkling of his facial skin. Other associated symptoms were seborrhea, acne, clubbed fingers, and occasional minimal knee joint pain. Histopathology results showed thickened dermis, fibrosis of the papillary dermis and around the folliculosebaceous units, hyalinized collagen bundles involving the fibrous trabeculae of the subcutis, and prominence of sebaceous and eccrine glands. The patient was given oral isotretinoin at 0.5 mg/kg/day, underwent fractional CO~ laser f?r rhytides and large pores, and given botuhnum toxin A injection (total of 16 U) on 5 sites at the glabellar region. The patient was referred to plastic surgery for frontal rhytidectomy, and orthopedic surgery for management of joint pains.
RESULTS:The patient noted 80% improvement from baseline.
CONCLUSION:Treatment of pachydermoperiostosis is mainly symptomatic and requires a multi-spec~alty approach. Because of its rarity, treatment options for pachydermoperiostosis have yet to_ be standardized. In this particular case, all available options in the institution were utilized which led to satisfaction of the patient of the outcome. - Full text:mask of furrow.pdf
