Rhytidectomy for pachydermoperiostosis

Sir,

Pachydermoperiostosis, a rare genetic disorder, presents with various cutaneous manifestations such as thickened skin thrown into folds and furrows, thickened eyelids with mechanical ptosis and seborrhea.¹ The furrows on the forehead give an appearance of premature aging resulting in significant cosmetic morbidity. There are no successful medical treatment options for improving the facial appearance in these patients and therefore, it remains a therapeutic challenge.²

Rhytidectomy on the forehead furrows has been performed rarely to improve the facial features in patients with pachydermoperiostosis.³ The procedure of rhytidectomy is carried out as follows: The furrows to be excised are marked [Figure 1]. Taking sterile aseptic precautions, local anesthesia containing lignocaine 2% with adrenaline 1:100,000 is infiltrated around the central forehead furrows and other horizontal and longitudinal furrows using a 26-gauge needle. After anesthesia, an incision is made on the upper and lower furrow, followed by excision of central forehead skin (along the furrows). Other furrows are excised more selectively sparing the adjoining skin. The furrows are excised up to the level of the subcutaneous tissue [Video 1]. After hemostasis and undermining [Video 2], the wound is closed in layers with Vicryl (polyglactin 910) 4–0 suture in the subcuticular plane and Prolene (polypropylene) 5–0 suture in the skin layer [Video 3]. The suture line is placed in the furrow itself. Oral antibiotics are prescribed after the surgery. The sutures may be removed on the seventh post-operative day. Significant improvement can be achieved in facial appearance after surgery.

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Figure 1:

Pictorial representation of incision placement

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We report a case of pachydermoperiostosis who experienced excellent transformation of his facial features after three sessions of rhytidectomy on his forehead. A 30-year-old male had complaints of thickening of skin over the scalp (cutis verticis gyrata), face (coarse facies and deep furrows and mechanical ptosis), both hands, feet and shin with associated clubbing, palmoplantar hyperhidrosis and joint pain and swelling in the bilateral knee and ankle joints for the past nine years. Laboratory investigations including hemogram, liver and renal functions, thyroid function test, rheumatoid factor, anti-nuclear antibody, anti-cyclic citrullinated peptide, oral glucose tolerance test and insulin-like growth factor-1 levels were normal. With a diagnosis of pachydermoperiostosis, he received hydroxychloroquine 200 mg twice daily for six months with minimal improvement. Thereafter, the treatment was changed to methotrexate 25 mg a week and naproxen one gram daily followed by the addition of colchicine one milligram daily which did not result in improvement in his facial appearance, atthough joint pains improved significantly. When he presented to us [Figure 2], we decided to perform rhytidectomy in view of non-responsiveness to the medical treatment. We performed three sessions of rhytidectomy of deep and prominent furrows which were present over the forehead under local anesthesia over a period of 12 months. Overall, the patient was satisfied with the outcome of improved facial features,which gave him a younger look [Figure 3].

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Figure 2:

Deep furrows over the forehead of the patient with pachydermoperiostosis (baseline)

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Figure 3:

Significant improvement in the facial appearance of the patient (after three sessions of serial rhytidectomy)

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In conclusion, the ease of performance and esthetic cosmetic results make rhytidectomy an effective therapeutic option for patients with pachydermoperiostosis.