Losing by a nose: diagnostic and therapeutic challenges of the trigeminal trophic syndrome.

Peripheral or central damage to the trigeminal nerve is a relatively common occurrence, whether due to cerebrovascular disease, viral infections (such as varicella-zoster), idiopathic Bell palsy, or surgical ablation of the Gasserian ganglion to treat neuralgic pain. (1) Rarely, such injury can be complicated by the development of a triad of anesthesia, paresthesias, and crescentic ala nasi ulceration known as the trigeminal trophic syndrome (TTS). (2) The distinctive nasal ulceration of the TTS is self-induced, resulting from repetitive manipulation in response to the anesthesia and/or paresthesias. It is important for healthcare practitioners to be aware of this well-described syndrome because mistaking the ulcerative lesions for either invasive infection or autoimmune disease (eg, Wegener granulomatosis) may result in inappropriate, and potentially damaging, therapy with systemic antimicrobials or immunosuppressive drugs. (1,3)

The review of TTS presented by Setyadi et al in this issue of the Southern Medical Journal is a welcome addition to the literature. (4) Although more than 100 cases of TTS have been published, it is a disease seldom addressed in internal medicine journals. Setyadi et al present an interesting case of TTS and review important aspects of the syndrome with clinically useful tables, outlining the differential diagnosis of nasal ulceration and highlighting differences between TTS and factitial disorders. A strength of their work is the inclusion of instructive photographs of the TTS lesion and the histologic biopsy specimens.

Although the case presented within their review is a "textbook" example of TTS in its clinical and microscopic features, it is atypical because the development of nasal ulceration occurred nearly three decades after the presumed initial insult, which was either a right-sided Bell palsy or a cerebrovascular accident, or both. (4) Though TTS may not occur for months to years after trigeminal injury, 30 years is an unusually long latency and suggests that a superimposed process provoked the syndrome. Indeed, the authors argue that the recent onset of dementia may have unmasked or fostered the repetitive manipulation of the nasal ala that culminated in tissue destruction. Whether a recent varicella-zoster reactivation in the trigeminal distribution may have occurred remains a possibility, although the tissue pathology did not disclose evidence of shingles, such as multinucleated giant cells. Data are lacking regarding immunofluorescence staining or PCR analysis of the biopsy tissue for varicella zoster virus. It is assumed that stains for other possible microbial etiologies were negative. In addition, it is unclear if neurologic imaging was performed to rule out an additional stroke.

The case described by Setyadi et al emphasizes a frustrating aspect of treating TTS: that patients can rarely be stopped from manipulating their ulcers. As the pathophysiological basis for this syndrome is not fully understood, therapeutic options are limited. The most successful strategies are, at best, anecdotal, not having been rigorously tested. Behavioral modification and physically protecting the ulcer from manipulation are mainstays. Nevertheless, a myriad of pharmacological therapies aimed at diminishing paresthesias and compulsive behaviors have been applied with varied success, including amitriptyline, carbamazepine, chlorpromazine, diazepam, pimozide, vitamin B supplements, and clonazepam. (1) In this report, Setyadi et al unsuccessfully prescribed a selective serotonin reuptake inhibitor for their patient.

The Southern Medical Journal audience is encouraged to read the review by Setyadi and colleagues and add the TTS to the list of causes of facial ulceration, particularly involving the ala nasi. Once a definitive diagnosis of TTS is established, an interdisciplinary team of healthcare providers, including a primary care provider and either a psychiatrist or psychologist with expertise in compulsive behavior modification, might optimize the chances for treatment to succeed.

References

1. Monrad SU, Terrell JE, Aronoff DM. The trigeminal trophic syndrome: an unusual cause of nasal ulceration. J Am Acad Dermatol 2004;50:949-952.

2. Weintraub E, Soltani K, Hekmatpanah J, et al. Trigeminal trophic syndrome. A case and review. J Am Acad Dermatol 1982;6:52-57.

3. Datta RV, Zeitouni NC, Zollo JD, et al. Trigeminal trophic syndrome mimicking Wegener's granulomatosis. A case report with a review of the literature. Ann Otol Rhinol Laryngol 2000;109:331-333.

4. Setyadi HG, Cohen PR, Schulze KE, et al. Trigeminal trophic syndrome. South Med J 2007;100:45-48.

David M. Aronoff, MD

From the Department of Internal Medicine, Division of Infectious Diseases, the University of Michigan Health System, Ann Arbor, MI.

Reprint requests to David M. Aronoff, MD, 5220-D MSRB III, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-0640. Email: [e-mail]

Accepted August 2, 2006.