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A 66-year-old man presented with multiple progressive ulcers on the right side of his face that had developed over the past 3 months (Figure, A). He had also experienced a burning sensation on the right side of his face, but denied headache, shoulder, or jaw pain and visual disturbance. Neurological examination revealed hypoesthesia of the right side of the face, while cold-warm sensation and motor function were intact. The erythrocyte sedimentation rate was 82 mm per hour (normal value, less than 15 mm per hour) and the serum C–reactive protein concentration was 107 mg/L (normal value, less than 5 mg/L). Chest radiography showed bilateral perihilar infiltrates consistent with pneumonia. About 2 weeks before the ulcerations developed, the patient had been admitted to the hospital for unstable angina, and coronary angiography was performed. Immediately after the angiography, the patient developed left-sided hemiplegia and hemianopsia. Magnetic resonance imaging of the brain revealed an extensive ischemic posterior circulation stroke involving the right occipital lobe, lower cerebellar peduncle, and pontomedullary boundary (Figure, B).
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C. Trigeminal trophic syndrome
As the patient was unaware of the self-inflicted nature of the injuries, he was advised to wear cotton gloves to prevent further skin damage due to unintentional scratching, and the ulcers were treated with daily occlusive antiseptic dressings. All lesions healed almost completely within 7 weeks, while hypoesthesia and paresthesia of the right face also resolved. The pneumonia responded to antibiotic therapy, while the erythrocyte sedimentation rate and C-reactive protein levels normalized.
Trigeminal trophic syndrome is a rare disease following peripheral or central damage to the fifth cranial nerve.1,2 Unilateral ulcers in the sensory distribution of the trigeminal nerve are characteristic.2 The ulcers are most frequently located in the area supplied by the maxillary division of the trigeminal nerve, that is, on the nasal ala, cheek, and upper lip, sparing the nasal tip.2 Hypoesthesia is often combined with paresthesia in the form of a burning, crawling, and itching sensation. As a result, self-inflicted lesions develop, secondary to unconscious traumatic rubbing and scratching.2 Like skin ulcers from external trauma in general, they typically have sharply demarcated, pauci-inflammatory borders and can be geometrically shaped. There is also the lack of a primary lesion such as pustule, vesicle, or inflammatory papule.
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Beke Esther Linnemann, Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, Lübeck, Germany (firstname.lastname@example.org).
Published Online: July 19, 2021. doi:10.1001/jamaneurol.2021.2236
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Ewan A. Langan, MD, University of Lübeck, Lübeck, Germany, for critically reading the manuscript. He was not compensated for his contribution to this work.
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