Trigeminal trophic syndrome secondary to recurrent meningioma
Authors: Christine C. Yang, Whitney L. Tolpinrud, Marc E. Grossman
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: A 45-year-old man with a history of childhood acute lymphocytic leukemia and radiation-induced bilateral recurrent meningiomas requiring resection in 2001 and resection and Gamma-Knife in 2010 presented with a 4-week history of unexplained facial ulcerations followed by the development of headaches. He reported waking with “bloody lesions” on his face, which were preceded by facial pain he rated on a scale of 1 to 10 as an 8. He denied pruritus and picking his face. His headaches were associated with left-sided photophobia and facial numbness. Physical exam revealed a red, ulcerated, indurated left tragus. Scattered 5- to 10-mm annular pink atrophic scars with peripheral hyperpigmentation were seen on the left cheek, forehead, and nose (Fig 1). A 2- x 1-cm superficial ulceration within a well-healed surgical scar and a 1.2-cm x 6-mm crescentic superficial ulceration on the left lateral nose were noted. Dried blood was noted under his fingernails. Neurologic exam revealed diminished sensation to touch, pinprick, and temperature on the left side of the face. Complete blood count with differential, complete metabolic profile, and liver function tests were normal. Skin biopsy of the left tragus showed normal skin without evidence of malignancy or significant inflammation. Brain magnetic resonance imaging study showed a left middle cranial fossa meningioma with interval enlargement compared with the previous year with involvement of the ipsilateral trigeminal nerve (Fig 2). A right posterior clinoid meningioma, unchanged from the previous study, was also present. Trigeminal trophic syndrome (TTS) involving all 3 branches of the trigeminal nerve was diagnosed. TTS, also called trigeminal neurotrophic ulceration, is a rare condition occurring after damage to the trigeminal nerve and presents with unilateral anesthesia with crescentic ulceration classically involving the ala nasi. Ulceration is self-induced secondary to paresthesias and patients are often unaware of self-mutilation. Causes of trigeminal nerve damage leading to TTS include trigeminal ablation, infarction, vertebrobasilar insufficiency, acoustic neuroma, astrocytoma, meningioma, spinal cord degeneration, Mycobacterium leprae neuritis, herpes zoster, syringobulbia, postencephalitic parkinsonism, and trauma.1Monrad S.U. Terrell J.E. Aronoff D.M. The trigeminal trophic syndrome: an unusual cause of nasal ulceration.J Am Acad Dermatol. 2004; 50: 949-952Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar Lesions of TTS most commonly occur in the second trigeminal division dermatome, followed by the first and third.2Garza I. The trigeminal trophic syndrome: an unusual cause of face pain, dysaesthesias, anaesthesia and skin/soft tissue lesions.Cephalalgia. 2008; 28: 980-985Crossref PubMed Scopus (31) Google Scholar One case of TTS involving all 3 branches of the trigeminal nerve in the context of surgical resection for meningioma has been reported.3Luksić I. Luksić I. Sestan-Crnek S. Virag M. Macan D. Trigeminal trophic syndrome of all three nerve branches: an underrecognized complication after brain surgery.J Neurosurg. 2008; 108: 170-173Crossref PubMed Scopus (15) Google Scholar Another report described a patient in whom trigeminal sensory dysfunction developed 1 year after meningioma excision and subsequently ala nasi ulceration developed 6 weeks after a third surgery.4Weintraub E. Soltani K. Hekmatpanah J. Lorincz A.L. Trigeminal trophic syndrome. A case and review.J Am Acad Dermatol. 1982; 6: 52-57Abstract Full Text PDF PubMed Scopus (76) Google Scholar Trigeminal dysfunction causing facial numbness was reported in 11% of patients who underwent surgery for cranial base meningiomas.5Westerlund U. Linderoth B. Mathiesen T. Trigeminal complications arising after surgery of cranial base meningiomas.Neurosurg Rev. 2012; 35: 203-209Crossref PubMed Scopus (2) Google Scholar Nerve injury was attributed in some patients to intraoperative dissection within the cavernous sinus, tumor infiltration, or gross injury to the nerve distal to the cavernous sinus; 42% of patients had no identifiable cause. We report a case of TTS involving V1-V3 related to a recurrent meningioma. Although our patient had a history of surgical resection and Gamma-Knife therapy, which could have caused trigeminal nerve damage, the association with additional neurologic symptoms and the evidence visualized on neuroradiologic imaging suggest recurrent disease as the etiology of the TTS. Interestingly, the appearance of skin lesions preceded the development of headaches, suggesting that TTS can indicate neurologic disease before the development of other neurologic symptoms.