The authors describe the case of a 14-year-old girl who experienced progressive hemifacial atrophy at the age of three-and-a-half years. The patient's early age at appearance of the condition resulted not only in atrophy of skin, subcutaneous tissue, and muscles, but also in very severe deformation of the right side of the face due to disorders in growth of cartilaginous and osseous tissue. Changes in her brain case and craniofacial skeleton included enophthalmos, underdevelopment of the eyelids, nose, hard plate, and dental process of the jaw including the premolar teeth, and a lack of the second molar. These changes caused an irregular line of bite. The differential diagnosis excluded hemifacial microsomy and sclerodermal hemifacial atrophy.
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