Papillon-Lefèvre Syndrome: A Case Report

Abstract

Takayasu's arteritis (TA) is a major chronic vasculitis disorder that its etiology is unknown. Patients are mostly Asian women who often show nonspecific symptoms such as fever, myalgia, arthralgia, weight loss, and anemia. The report relates to a 17-year-old girl suffering from complaints, permanent and uncontinental pain starting from a month earlier, with loss of appetite and weight loss (4 kg), and night sweats. She had no diarrhea or gastrointestinal symptoms but had a pain in the shoulder and chest area since the last 5 months. She got better after seeing a physician and receiving supplements. She had a history of pain in the ear from the last five months leading to otitis, and was treated as a case of brucellosis with a score of 1.20 in the Coombs Wright test. According to the physical examination findings, the patient's left, radial, ulnar, and proximal pulses, and blood pressure were unexplained, and in the supraclavicular region of the left and the umbilical region, bruit was heard and the shape of the left nail was changed. Laboratory tests and imaging were performed for the patient, and after angiography, the left subclavian artery stenosis was detected. Given the age and sex of the patient and the results obtained, she was diagnosed with Takayasu's arteritis.