Managing Orofacial Changes in Scleroderma

CARLSBAD, California — In 2003, researchers asked 303 patients with systemic sclerosis (scleroderma) what bothered them most about their disease from an aesthetic standpoint: Orofacial features, such as thin lips and mouth furrows, or non-facial features, such as fingertip ulceration and waxy changes to the skin.

Respondents expressed significant concern about specific orofacial features, including thin lips (73%), mouth furrows (80%), loss of facial lines (68%), and a smaller, tighter mouth (77%).

“Patients with systemic sclerosis may have loss of vermilion lip, microstomia, and perioral rhytids,” Kathleen Cook Suozzi, MD, who directs the Aesthetic Dermatology Program at Yale University School of Medicine, New Haven, Connecticut, said at the Controversies and Conversations in Laser and Cosmetic Surgery annual symposium. “How can we address these changes for our patients?”

Recent research has shown that hyaluronidase injections can help improve orofacial changes commonly experienced by patients with scleroderma. In 2019, researchers in Alabama reported the case of a 53-year-old woman treated with hyaluronidase for scleroderma-induced microstomia. After four visits over 7 months and a total hyaluronidase dose of 470 IU, the patient reported an improved Mouth Handicap in Systemic Sclerosis (MHISS) score (38 of 48); subjective improvement of symptoms, including greater ease in eating and undergoing dental treatment; and improved mouth closure.

In 2023, researchers published a cohort study of four women between the ages of 43 and 61 with autoimmune sclerosing conditions that resulted in oral microstomia. Following hyaluronidase injections, all improved in mouth opening capacity and MHISS, with change stabilizing between three and five treatments. More recently, in a study pending publication in JAAD Case Reports, Suozzi and colleagues retrospectively evaluated 12 women with scleroderma who received between 150 and 300 units of hyaluronic acid (HA) filler for microstomia between 2020 and 2023. Of the 12 women, 58% had diffuse disease, and 42% had limited disease. Overall, oral aperture width increased by 0.65 cm (P =.0027) and oral aperture height increased by 0.88 cm (P <.0001). “In general, patients needed three to four treatments to reach peak effect, and then they reached a plateau,” Suozzi said. “It wasn’t that the treatment wasn’t working anymore, but it was because their oral aperture had gotten to a size of around 5 cm, which is clinically normal. Interestingly, we found that if the patient’s disease flared and their microstomia started to return, when you rechallenged them, they continued to respond. So, patients can continue to use this treatment over time.”

In a separate case series of seven patients, Suozzi and colleagues prospectively evaluated the effect of HA soft tissue filler with Restylane Silk for lip augmentation. Study participants experienced statistically significant increases in the difference between pre- and postinjection fullness in both upper and lower lips. Also, the mean posttreatment score fell between “much improved” (2) and “improved” (3) on both the Investigator Global Aesthetic Improvement Scale and the Subject Global Aesthetic Improvement Scale.

Suozzi recommends using nerve blocks for injecting HA filler or hyaluronidase in patients with scleroderma and minimizing the injection points. “Initially, we were using 30% lidocaine preparations around the mouth for an hour before the procedure, and patients were still having pain, so now we use nerve blocks,” she said. “For hyaluronidase, we do perform a test dose of 75-100 units, usually in the commissure. It’s amazing how well it works; people will usually come back after their test dose and have improvements in their measurements. This is a really easy treatment to perform, and I think it can be done in the office of a general dermatologist. There is concern about cross-reactivity with bee venom, so you want to ask patients about that.”

Suozzi reported having no relevant financial relationships.