Imaging in Headache Medicine
Trigeminal trophic syndrome: A rare cause of chronic facial pain and skin ulcers
Cephalalgia 2015, Vol. 35(7) 636 ! International Headache Society 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0333102414547140 cep.sagepub.com
Erin Golden1, Carrie E Robertson1, John J Moossy2, Paola Sandroni1 and Ivan Garza1 Date received: 27 May 2014; revised: 8 July 2014; 14 July 2014; accepted: 20 July 2014
Figure 1. Anterior (a) and inferior (b) view of patient’s face, showing scattered ulcers in the right V1–3 distributions. Note loss of lateral nasal ala.
A 25-year-old female presented with chronic right-sided facial pain and ulcers that had gradually developed over four years. Symptoms began after the last of four microvascular decompressions and partial trigeminal nerve section for trigeminal neuralgia. Investigations including biopsy for neoplastic, infectious, and inflammatory conditions were unrevealing. She was diagnosed with trigeminal trophic syndrome (TTS), a consequence of trigeminal nerve injury. This condition was originally described as ‘‘trigeminal neuropathy with nasal ulceration.’’ TTS consists of pain, anesthesia and ulcers in the trigeminal nerve distribution (1–4) (see Figure 1). Although the pathogenesis of TTS remains unknown, a large series of 99 cases has suggested that self-inflicted trauma of the paresthetic skin could be responsible for the lesions and therefore the habit should be abandoned (3). Pain is often refractory to multiple opiate and neuropathic pain medications (1–4). Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest None declared. References 1. Spillane JD and Urich H. Trigeminal neuropathy with nasal ulceration: Report of two cases and one necropsy. J Neurol Neurosurg Psychiatry 1976; 39: 105–113. 2. Garza I. The trigeminal trophic syndrome: An unusual cause of face pain, dysaesthesias, anaesthesia and skin/ soft tissue lesions. Cephalalgia 2008; 28: 980–985. 3. Maaßen D and Voigtla¨nder V. Das neurotrophe Trigeminus-Syndrom. Akt Dermatol 1990; 16: 3–6. 4. Monrad SU, Terrell JE and Aronoff DM. The trigeminal trophic syndrome: An unusual cause of nasal ulceration. J Am Acad Dermatol 2004; 50: 949–952.
1
Department of Neurology, Mayo Clinic College of Medicine, USA Department of Neurological Surgery, University of Pittsburgh Medical Center, USA 2
Corresponding author: Ivan Garza, Department of Neurology, Mayo Clinic College of Medicine, 200 First St. SW, Rochester, MN 55905, USA. Email: [email protected]
Copyright of Cephalalgia (Sage Publications Ltd.) is the property of Sage Publications, Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Trigeminal trophic syndrome: A rare cause of chronic facial pain and skin ulcers
Cephalalgia 2015, Vol. 35(7) 636 ! International Headache Society 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0333102414547140 cep.sagepub.com
Erin Golden1, Carrie E Robertson1, John J Moossy2, Paola Sandroni1 and Ivan Garza1 Date received: 27 May 2014; revised: 8 July 2014; 14 July 2014; accepted: 20 July 2014
Figure 1. Anterior (a) and inferior (b) view of patient’s face, showing scattered ulcers in the right V1–3 distributions. Note loss of lateral nasal ala.
A 25-year-old female presented with chronic right-sided facial pain and ulcers that had gradually developed over four years. Symptoms began after the last of four microvascular decompressions and partial trigeminal nerve section for trigeminal neuralgia. Investigations including biopsy for neoplastic, infectious, and inflammatory conditions were unrevealing. She was diagnosed with trigeminal trophic syndrome (TTS), a consequence of trigeminal nerve injury. This condition was originally described as ‘‘trigeminal neuropathy with nasal ulceration.’’ TTS consists of pain, anesthesia and ulcers in the trigeminal nerve distribution (1–4) (see Figure 1). Although the pathogenesis of TTS remains unknown, a large series of 99 cases has suggested that self-inflicted trauma of the paresthetic skin could be responsible for the lesions and therefore the habit should be abandoned (3). Pain is often refractory to multiple opiate and neuropathic pain medications (1–4). Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest None declared. References 1. Spillane JD and Urich H. Trigeminal neuropathy with nasal ulceration: Report of two cases and one necropsy. J Neurol Neurosurg Psychiatry 1976; 39: 105–113. 2. Garza I. The trigeminal trophic syndrome: An unusual cause of face pain, dysaesthesias, anaesthesia and skin/ soft tissue lesions. Cephalalgia 2008; 28: 980–985. 3. Maaßen D and Voigtla¨nder V. Das neurotrophe Trigeminus-Syndrom. Akt Dermatol 1990; 16: 3–6. 4. Monrad SU, Terrell JE and Aronoff DM. The trigeminal trophic syndrome: An unusual cause of nasal ulceration. J Am Acad Dermatol 2004; 50: 949–952.
1
Department of Neurology, Mayo Clinic College of Medicine, USA Department of Neurological Surgery, University of Pittsburgh Medical Center, USA 2
Corresponding author: Ivan Garza, Department of Neurology, Mayo Clinic College of Medicine, 200 First St. SW, Rochester, MN 55905, USA. Email: [email protected]
Copyright of Cephalalgia (Sage Publications Ltd.) is the property of Sage Publications, Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
