Journal of the Philippine Medical Association 2021;100(1):51-57
The mask of furrow: A case of a 28-year-old Filipino male with pachydermoperiostosis.
Precious Imam 1 ; Elizabeth P. Prieto ; Vilma Pelino ; Milali Torres ; Monique Lianne Lim Ang
Affiliations
Keywords
pachydermoperiostosis; primary hypertrophic osteoarthropathy
Country
Philippines
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.
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