50% of affected individuals develop dental abnormalities such as delayed eruption, dental root exposure, or resorption of the dental roots on the affected side. 35% have difficulty or inability to normally open the mouth or other jaw symptoms, including temporomandibular joint disorder and spasm of the muscles of mastication on the affected side. 25% experience atrophy of one side of the upper lip and tongue. Causes
The fact that some people affected with this disease have circulating antinuclear antibodies in their serum supports the theory that Parry–Romberg syndrome may be an autoimmune disease, specifically a variant of localized scleroderma. Several instances have been reported where more than one member of a family has been affected, prompting speculation of an autosomal dominant inheritance pattern. However, there has also been at least one report of monozygotic twins in which only one of the twins was affected, casting doubt on this theory. Further, the National Organization for Rare Disorders has stated there is currently no evidence that Parry–Romberg syndrome is genetic or that it can be passed on to children. Various other theories about the cause and pathogenesis have been suggested, including alterations in the peripheral sympathetic nervous system (perhaps as a result of trauma or infection involving the cervical plexus or the sympathetic trunk), as the literature reported it following sympathectomy, disorders in migration of cranial neural crest cells, or chronic cell-mediated inflammatory process of the blood vessels. It is likely that the disease results from different mechanisms in different people, with all of these factors potentially being involved.
In addition to its helpful effects in treating AU, it can have side effects that can be very serious, such as severe dermatitis.Topical and intralesional corticosteroids, such as clobetasol propionate, have also shown to be an effective treatment for AT and AU patients. A controlled study comprising 28 patients found positive terminal hair growth in eight of the patients (28.5%) using a 0.05% clobetasol propionate ointment. This is very similar to the results obtained from immunotherapy treatment trials. Additionally, studies suggest that intralesional applications are much more effective than topical applications of steroids. However, the main side effect is increased risk of cutaneous atrophy at the site of treatment; folliculitis is also an occasional complication.Janus kinase inhibitors, previously used in the treatment of cancer and other diseases, such as arthritis, have successfully shown to be effective in the initial trials of treatment for alopecia patients. Multiple cases of treatments have been successful, one of them being of a 22-year-old man with a history of AU and atopic dermatitis (AD). This man was treated with JAK inhibitor tofacitinib, and after ten months, he experienced hair regrowth on all of his affected body parts and subsequent improvement of his AD. Current research and findings suggest that systemic JAK inhibitors eliminate and prevent the development of AA, while topical JAK inhibitors promote hair regrowth and reverse the established disease. Many clinical trials are ongoing involving JAK inhibitors such as ruxolitinib and tofacitinib. See also
Alopecia areata
Alopecia totalis

References


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