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