(Last Updated On: October 30, 2018)

Authors: Patricia Anne Nicole O. Ramirez, MD, Cyryl Rae Benjamine S. Rejuso-Kalbit, MD, Barbara Ellen T. Marcelo, MD, Milali R. Torres, MD, FPDS

Abstract

Introduction: Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a rare, genetic syndrome with familial and idiopathic forms characterized by progressive joint effusion associated with pachydermia, periostosal proliferation of the long bones, and finger clubbing. This condition presents with distinctive thickening and furrowing of the skin of the scalp, forehead and cheeks, enlargement of the distal parts of the extremities, excessive sweating, and severe seborrhea of the scalp and face.

Case Summary: This is a case of a 27-year old male with a 10-year history of acne, seborrhea, joint pains, enlargement of the wrists, fingers, knees and ankles, and palmoplantar hyperhidrosis. He also noted the development of thickened and furrowed skin of the forehead, cheeks and scalp. Histopathology results were consistent with pachydermoperiostosis. The patient responded well to oral isotretinoin at a dose of 0.5mg/kg/day. He also underwent forehead rhytidectomy under the plastic surgery service, with noted improvement of the furrows on his forehead. He is maintained on oral retinoids.

Conclusion: The typical course of this disease is self-limited as it appears in adolescence, progresses for a number of years, and stabilizes, without diminishing life expectancy. However, once disease has stabilized, patients are left with significant cosmetic morbidity. As physicians it is always our aim to improve the patient’s quality of life. In cooperation with other subspecialties, I believe that it is possible to address the varying issues of our patients. In our case, treatment has been mainly focused on the control of sebum production and cosmetic improvement by plastic surgery.

 

Citation

 

Keywords

pachyderma, pachydermoperiostosis, primary hypertrophic osteoarthropathy

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