Trigeminal trophic syndrome.Abstract: Ulceration of the nose may be inadvertently induced by the patient. Although trigeminal trigeminal /tri·gem·i·nal/ (tri-jem´i-n'l) 1. triple. 2. pertaining to the trigeminal (fifth cranial) nerve. 3. pertaining to trigeminy. tri·gem·i·nal adj. trophic trophic /tro·phic/ (tro´fik) (trof´ik) pertaining to nutrition. troph·ic adj. Of, relating to, or characterized by nutrition. syndrome is an uncommon cause of chronic ulcers, healthcare providers should consider the possibility of this disorder when encountering a patient with nasal ulcerations Ulcerations Breaks in skin or mucous membranes that are often accompanied by loss of tissue on the surface. Mentioned in: Hypersplenism . Trigeminal trophic syndrome most commonly occurs in older women following therapy for trigeminal neuralgia Trigeminal Neuralgia Definition Trigeminal neuralgia is a disorder of the trigeminal nerve (the fifth cranial nerve) that causes episodes of sharp, stabbing pain in the cheek, lips, gums, or chin on one side of the face. . The ulcers usually involve the nasal ala and paranasal areas. The clinical vignette of a man with a self-induced nasal ulcer secondary to trigeminal trophic syndrome, which was initially suspected to be skin cancer, is presented. Since nasal ulcerations can be secondary to other conditions, a lesional biopsy should be performed to exclude other diagnoses when trigeminal trophic syndrome is entertained. In addition to trigeminal trophic syndrome, the differential diagnosis differential diagnosis n. Determination of which one of two or more diseases with similar symptoms is the one from which the patient is suffering. Also called differentiation. of conditions that can cause nasal ulcers include factitial disorders with self-induced ulcerations (such as dermatitis artifacta and neurotic excoriations), granulomatous granulomatous /gran·u·lom·a·tous/ (-lom´ah-tus) containing granulomas. Granulomatous Resembling a tumor made of granular material. conditions, infectious diseases, malignancy, and pyoderma gangrenosum pyoderma gan·gre·no·sum n. A chronic skin disease, usually of the trunk, characterized by large spreading ulcers. pyoderma gangrenosum . Treatment of trigeminal trophic syndrome requires prevention of digital manipulation of the lesion--either by occluding contact with the ulcer, initiating psychotropic psychotropic /psy·cho·tro·pic/ (si?ko-tro´pik) exerting an effect on the mind; capable of modifying mental activity; said especially of drugs. psy·cho·tro·pic adj. medication, or both. Psychiatric and/or pharmacologic intervention should be considered to reduce or resolve further habitual self-inflicted injury before surgical intervention. Key Words: Alzheimer, basal, cancer, carcinoma, cutaneous cutaneous /cu·ta·ne·ous/ (ku-ta´ne-us) pertaining to the skin. cu·ta·ne·ous adj. Of, relating to, or affecting the skin. Cutaneous Pertaining to the skin. , dementia, factitial, factitious factitious /fac·ti·tious/ (fak-tish´-us) artificially induced; not natural. fac·ti·tious adj. Produced artificially rather than by a natural process. , self-induced, nasal, skin, squamous, stroke, trigeminal, trophic, ulcer ********** Self-induced ulceration can occur at any site, including the nose. Nasal ulceration may be caused by several conditions, including trigeminal trophic syndrome (Table 1). (1-11) The epidemiology, clinical manifestations, differential diagnosis and laboratory evaluations are reviewed. The mechanisms of pathogenesis for trigeminal trophic syndrome and the therapeutic modalities for treating this condition are also reviewed. Trigeminal Trophic Syndrome: Vignette A 91-year-old man with an ulcer on his right nose for 9 months presented for evaluation of the lesion (Fig. 1). His past medical history was significant for Alzheimer disease, right-sided Bell palsy 34 years previously, and a stroke 30 years prior, causing a right-sided facial hemiparesis hemiparesis /hemi·pa·re·sis/ (-pah-re´sis) paresis affecting one side of the body. hem·i·pa·re·sis n. Slight paralysis or weakness affecting one side of the body. . He had been repeatedly observed to pick at his nose with his fingers. Clinical examination of the nose revealed a 2.5 X 2.8-cm firm nodular nodular marked with, or resembling, nodules. nodular dermatofibrosis see dermatofibrosis. nodular episcleritis see nodular fasciitis (below). nodular fasciitis a firm painless nodular swelling, 0. plaque with a full-thickness ulcer that was surrounded by a flesh-colored pearly border (Fig. 2A). Part of the right nasal ala was absent (Fig. 2B). The initial clinical diagnosis was basal cell carcinoma basal cell carcinoma n. A slow-growing, locally invasive, but rarely metastasizing neoplasm of the skin derived from basal cells of the epidermis or hair follicles. Also called basal cell epithelioma. . A lesional biopsy, which included the center and the border of the ulcer, was performed to establish the diagnosis (Fig. 3). Histology revealed hemorrhagic Hemorrhagic A condition resulting in massive, difficult-to-control bleeding. Mentioned in: Hantavirus Infections hemorrhagic pertaining to or characterized by hemorrhage. crust containing bacteria overlying overlying suffocation of piglets by the sow. The piglets may be weak from illness or malnutrition, the sow may be clumsy or ill, the pen may be inadequate in size or poorly designed so that piglets cannot escape. the hyperplastic epithelium at the peripheral portion of the specimen. Vascular proliferation and fibrosis were present in the dermis dermis: see skin. beneath the ulcer in the central portion of the lesion. Correlating the patient's history of dementia and stroke on the ipsilateral ipsilateral /ip·si·lat·er·al/ (ip?si-lat´er-al) situated on or affecting the same side. ip·si·lat·er·al adj. Located on or affecting the same side of the body. side of the nasal ulcer, the clinical features of the lesion, and the pathologic findings, the diagnosis of a self-induced nasal ulceration resulting from trigeminal trophic syndrome was made. It is probable that it was unmasked by advancing dementia-associated disinhibition dis·in·hi·bi·tion n. 1. A loss of inhibition, as through the influence of drugs or alcohol. 2. A temporary loss of an inhibition caused by an unrelated stimulus, such as a loud noise. of compulsive, habitual picking behavior. [FIGURE 1 OMITTED] Discussion Psychopharmacologic therapy was initiated using sertraline sertraline /ser·tra·line/ (ser´trah-len) a selective serotonin reuptake inhibitor used as the hydrochloride salt in the treatment of depression, obsessive-compulsive disorder, and panic disorder. , at a dose of 150 mg per day. There was no significant improvement in either the skin ulcer or the patient's compulsive picking at the lesion, making surgical reconstruction inappropriate. [FIGURE 2 OMITTED] Trigeminal trophic syndrome is an uncommon cause of chronic ulcers. This condition is seen mostly in the elderly, (4,12) with a mean age of 60 years and a median of 63 years. (6) Reported patients have ranged in age from 14 months to 93 years old. (6) This condition occurs more often in women, (4,12) with a female to male ratio of approximately 2:1. (5) [FIGURE 3 OMITTED] Clinical Manifestations The lesions typically present as crusted, (2,5,6,11,13) painless (2,5,6,13) crescent-shaped (1,5,6,11) ulcerations of the lateral nasal ala (Figs. 1 and 2). (1,4) The base may be covered by a blood crust and granulation tissue. (5) The affected site, predominantly the nasal ala, is innervated innervated adjective Containing or characterized by nerves by the first and second divisions of the trigeminal nerve, (2,5,6,12-14) most often where sensory innervation innervation /in·ner·va·tion/ (in?er-va´shun) 1. the distribution or supply of nerves to a part. 2. the supply of nervous energy or of nerve stimulation sent to a part. overlaps between the two divisions. (13,15) Bauer, (15) in 1966, theorized that the zone of overlap is affected with sensations of pruritus pruritus /pru·ri·tus/ (proo-ri´tus) itching.prurit´ic pruritus a´ni intense chronic itching in the anal region. pruritus hiema´lis xerotic eczema. or paresthesia paresthesia /par·es·the·sia/ (par?es-the´zhah) morbid or perverted sensation; an abnormal sensation, as burning, prickling, formication, etc. par·es·the·sia or par·aes·the·sia n. because of the change in the differential sensory input between the two trigeminal branches. However, the nasal tip is spared because of its pure medial ethmoidal ethmoidal pertaining to the ethmoid bone. enzootic ethmoidal tumor see enzootic ethmoidal tumor. ethmoidal foramen carries the ethmoidal nerve as it re-enters the cranial cavity. nerve innervation; yet, the surrounding nasal and medial cheek skin may be affected since they are innervated by sensory fibers from both the ophthalmic and maxillary max·il·lar·y adj. Of or relating to a jaw or jawbone, especially the upper one. n. A maxillar; a jawbone. maxillary (mak´siler´ē), adj divisions of the trigeminal nerve. The upper lip, (5,6,11) cheek, (2,5,6) temple, (2) scalp, (2,5,6) forehead, ear, palate, or jaw (4-6) may rarely be affected. Lesions in hair-bearing skin regions such as the scalp may result in secondary alopecia alopecia (ăl'əpē`shēə): see baldness. . (11,13) Trigeminal trophic syndrome is most commonly unilateral, (6) with the right side involved almost twice as often as the left. One case of bilateral involvement after bilateral trigeminal nerve ablation (6) and two patients with bilateral facial neurologic deficits and bilateral facial ulcers (5) have been reported. A fibrotic band originating from the nose pulling upwards results in a characteristic sneer. (4,5,16) The ulcer may be preceded or accompanied by ipsilateral nasal congestion, (1,5) burning, (1,2,4-6,12) pruritus, crawling sensation, (1,2,5,6,12,15) hearing loss, and/or absence of corneal reflex. (2,11,13) Other less common ocular findings in trigeminal trophic syndrome are neurotrophic keratitis keratitis Inflammation of the cornea (see eye). The conjunctiva may also be inflamed (keratoconjunctivitis). Depending on the cause, including dryness of the eye (from low tear production or inability to close the eye), chemical or physical injury, or certain , iritis iritis (īrī`tĭs), inflammation of the iris, the pigmented portion of the eye surrounding the pupil. The condition is sometimes associated with diabetes, with rheumatic diseases such as rheumatoid arthritis, and with infections such as , and corneal corneal pertaining to the cornea. See also keratitis, keratopathy. corneal anomaly includes microcornea, coloboma, megalocornea, dermoid, congenital opacity. corneal black body see corneal sequestrum (below). ulceration and opacification. (1,4) Trigeminal trophic syndrome is sometimes described as a triad of trigeminal anesthesia, facial paresthesia, and crescentic nasal alar ulceration. (1,4,5) The ipsilateral facial paresthesia (1,2,4,5,11,12,13,15) prompts touching, rubbing, scratching, or picking to alleviate the perceived irritation. (4-6,11,15,18) The ipsilateral trigeminal anesthesia causes a sensation of nasal congestion due to the perceived lack of passage of air. This results in the repeated manipulation of alar skin, which often becomes compulsive. (4,5,11,17,18) Most patients have insight upon presentation (2,3,6) while others will eventually (4) admit to painless self-manipulation. (5) Laboratory Evaluation Tissue biopsy is very important to rule out other conditions. The histologic findings of trigeminal trophic syndrome are not pathognomonic pathognomonic /pa·thog·no·mon·ic/ (path?ug-no-mon´ik) specifically distinctive or characteristic of a disease or pathologic condition; denoting a sign or symptom on which a diagnosis can be made. . They usually show ulceration, (2,4) acanthosis of the epidermis, (2) dermal dermal /der·mal/ (der´mal) pertaining to the dermis or to the skin. der·mal or der·mic adj. Of or relating to the skin or dermis. inflammatory changes, (4,11) and reactive vascular and connective tissue changes in the dermis. (13) Microscopic examination of the lesional biopsy from the patient described in the clinical vignette showed epithelial hyperplasia with vascular proliferation and fibrosis in the dermis (Fig. 3). In addition to tissue biopsy, lab testing such as angiotensin converting enzyme Noun 1. angiotensin converting enzyme - proteolytic enzyme that converts angiotensin I into angiotensin II angiotensin-converting enzyme, ACE peptidase, protease, proteinase, proteolytic enzyme - any enzyme that catalyzes the splitting of proteins into (ACE) for sarcoidosis Sarcoidosis Definition Sarcoidosis is a disease which can affect many organs within the body. It causes the development of granulomas. Granulomas are masses resembling little tumors. They are made up of clumps of cells from the immune system. , (1) c-antineutrophil cytoplasmic cytoplasmic pertaining to or included in cytoplasm. cytoplasmic inclusions include secretory inclusions (enzymes, acids, proteins, mucosubstances), nutritive inclusions (glycogen, lipids), pigment granules (melanin, lipofuscin, antibody (c-ANCA) to rule out Wegener granulomatosis, rapid plasma reagin Rapid Plasma Reagin (RPR) refers to a type of test that looks for non-specific antibodies in the blood of the patient that may indicate that the organism (Treponema pallidum) that causes syphilis is present. (RPR (Resilient Packet Ring) A packet-based protocol that provides fault tolerance and statistical multiplexing for the metropolitan and national SONET and Ethernet networks of the carriers. ) for syphilis, (1,4) Tzanck smear and viral culture to evaluate for herpes, (5) and other appropriate cultures and serologic tests to rule out other infectious causes should be considered. Differential Diagnosis The differential diagnosis for self-induced nasal ulceration includes factitial disorders, granulomatous conditions such as Wegener granulomatosis and sarcoidosis, various infectious diseases including bacterial, viral, fungal, and parasitic, malignancy such as basal cell carcinoma and squamous cell carcinoma squamous cell carcinoma n. A carcinoma that arises from squamous epithelium and is the most common form of skin cancer. Also called cancroid, epidermoid carcinoma. , and pyoderma gangrenosum (Table 1). It may be difficult to differentiate between trigeminal trophic syndrome and factitial disorders when a patient presents with a self-induced nasal ulcer. In the latter disorders, the neurologic history and the physical examination are usually normal. However, similar to trigeminal trophic syndrome, the microscopic examination of a lesional biopsy is also nonspecific nonspecific /non·spe·cif·ic/ (non?spi-sif´ik) 1. not due to any single known cause. 2. not directed against a particular agent, but rather having a general effect. nonspecific 1. . The treatment for factitial disorders is initially symptomatic; subsequently, psychiatric-based intervention may be incorporated to address the underlying problem (Table 2). If facial neurologic signs are present, the diagnosis of a factitial dermatosis dermatosis /der·ma·to·sis/ (der?mah-to´sis) pl. dermato´ses any skin disease, especially one not characterized by inflammation. is not likely. (5) Pathogenesis The pathogenesis of trigeminal trophic syndrome involves self-manipulation of the skin. (5,6,15,16,18,19) In 75% of trigeminal trophic syndrome patients, (2,5) this behavior develops following either surgical or chemical therapy for trigeminal neuralgia, such as surgical trigeminal ablation by rhizotomy rhizotomy /rhi·zot·o·my/ (ri-zot´ah-me) interruption of a cranial or spinal nerve root, such as by chemicals or radio waves. percutaneous rhizotomy of the dorsal trigeminal nerve root or alcohol injection, which disrupts the Gasserian ganglion. (1,2,4-6,11,12,14,16) Another common cause is stroke (1,4,6,12,13)--especially those which either affect the posterior cerebral circulation (2) or manifest as Wallenberg lateral medullary syndrome Lateral medullary syndrome (also called Wallenberg's syndrome and posterior inferior cerebellar artery syndrome) is a disease in which the patient has difficulty with swallowing or speaking or both owing to one or more patches of dead tissue (known as an . (6) Other causes of trigeminal trophic syndrome include sensory denervation denervation /de·ner·va·tion/ (de?ner-va´shun) interruption of the nerve connection to an organ or part. denervation of the trigeminal nerve (4) by intracranial intracranial /in·tra·cra·ni·al/ (-kra´ne-al) within the cranium. in·tra·cra·ni·al adj. Within the cranium. neoplasms, (2,4,5) intracranial surgery, (14) trauma, (12) postencephalitic Parkinsonism, syringobulbia, (5,6,11) craniotomy Craniotomy Definition Surgical removal of part of the skull to expose the brain. Purpose A craniotomy is the most commonly performed surgery for brain tumor removal. , (6) varicella varicella: see chicken pox. , (2) herpes simplex (2,4,5,20) or mycobacterial mycobacterial emanating from or pertaining to mycobacterium. mycobacterial granuloma may be caused by Mycobacterium tuberculosis (see cutaneous tuberculosis), M. infection. (2,4) The time between the trigeminal nerve injury and the onset of ulceration can vary from 2 weeks to 30 years, (6) with a median interval of 1 year. (5) In our patient, the onset of nasal ulcer was 30 years after the associated neurologic insult. Dementia has been suggested to prevent self-inhibition of compulsion, habitual manipulation, and nonintentional self-injury. Mental impairment has also been demonstrated to be an important factor in the development of trigeminal trophic syndrome. In 1985, Harper (19) noted that mental abnormality was described in the reports of 19% of the 31 trigeminal trophic syndrome patients he could find in the literature. Treatment Trigeminal trophic syndrome is difficult to treat. The primary purpose of management is to treat secondary problems, such as infection, and to prevent further trauma. (12) The initial step after diagnosis is local wound care, including topical and systemic antibiotics, if necessary. (1,2,4-6) Occlusive dressings, (2) finger bandages, protective gloves, nasal casts or prostheses Prostheses A synthetic object that resembles a missing anatomical part. Mentioned in: Microphthalmia and Anophthalmia have been used in an attempt to prevent digital manipulation. (1,4-6) Early investigators also noticed that vitamin B supplementation had some success in treating trigeminal trophic syndrome. (4-6) Some investigators emphasize the importance of educating the patient about the self-induced nature of the ulcers. (4-6) Psychotropic medications are commonly prescribed to prevent compulsive behavior elicited by the facial paresthesias Paresthesias A prickly, tingling sensation. Mentioned in: Autoimmune Disorders . (4,21) Carbamazepine carbamazepine /car·ba·maz·e·pine/ (kahr?bah-maz´e-pen) an anticonvulsant and analgesic used in the treatment of pain associated with trigeminal neuralgia and in epilepsy manifested by certain types of seizures. , initially at a dosage of 100 mg twice daily, (3) and increased to 200 mg twice daily if the patient tolerates the medication after 10 days, has been effective in some cases. (2,4,6) Other psychiatric medications--including diazepam diazepam /di·az·e·pam/ (di-az´e-pam) a benzodiazepine used as an antianxiety agent, sedative, antipanic agent, antitremor agent, skeletal muscle relaxant, anticonvulsant, and in the management of alcohol withdrawal symptoms. , amitriptyline hydrochloride, chlorpromazine chlorpromazine (klōrpräm`əzēn'), one of a group of tranquilizing drugs called phenothiazines that are useful in halting psychotic episodes. , (2,4-6) clonazepam clonazepam /clo·naz·e·pam/ (klo-naz´e-pam) a benzodiazepine used as an anticonvulsant and as an antipanic agent. clo·naz·e·pam n. , (4) and pimozide pimozide /pi·mo·zide/ an antipsychotic and antidyskinetic agent used in the treatment of Gilles de la Tourette's syndrome. pim·o·zide n. (22)--have occasionally been shown to be successful. Surgical treatment should only be considered for lesions that have started to heal with conservative wound care and after digital manipulation by the patient has ceased. (1) However, there has been a high recurrence rate of trigeminal trophic syndrome ulcers following surgical management. For example, ulcers recurred in 4 of 5 individuals from a series of trigeminal trophic syndrome patients following surgical repair of their lesions. (1) Postsurgical self-wounding may halt temporarily, but resume in the same area up to 5 years after repair. (1,5) Contralateral contralateral /con·tra·lat·er·al/ (-lat´er-al) pertaining to, situated on, or affecting the opposite side. con·tra·lat·er·al adj. innervated forehead flaps to prevent self-tampering have shown good results. (17,23) Other interventional therapies have also been successful in trigeminal trophic syndrome patients. Ipsilateral cervical sympathectomy Sympathectomy Definition Sympathectomy is a surgical procedure that destroys nerves in the sympathetic nervous system. The procedure is done to increase blood flow and decrease long-term pain in certain diseases that cause narrowed blood vessels. has also shown improvement in individuals. (4,11,12) Transcutaneous transcutaneous /trans·cu·ta·ne·ous/ (-ku-ta´ne-us) transdermal. trans·cu·ta·ne·ous adj. Transdermal. electrical stimulation to the area has also been effective for healing ulcers, (2,4,6) as long as treatment is continued. (24) Conclusion Trigeminal trophic syndrome is seen most commonly in the elderly as an ulcer on the nasal ala. The mechanism of the skin lesion is unintentional self-injury. It is important to rule out other causes of nasal ulceration, such as cancer or infection, when the diagnosis of trigeminal trophic syndrome is being considered. Although the pathologic features of trigeminal trophic syndrome are nonspecific, a biopsy of a nasal ulcer is helpful to exclude other conditions in the clinical differential diagnosis. Treatment of trigeminal trophic syndrome is initially directed at the local wound and addressing the underlying etiology for the syndrome. Management often includes psychopharmacologic agents. Once the nonintentional destructive behavior is resolved, surgical management may be considered. References 1. Tollefson TT, Kriet JD, Wang TD, et al. Self-induced nasal ulceration. Arch Facial Plast Surg 2004;6:162-166. 2. Hancox JG, Wittenberg GF, Yosipovitch G. A patient with nasal ulceration after brain surgery. Arch Dermatol 2005;141:796-798. 3. Bhushan M, Parry EJ, Telfer NR. Trigeminal trophic syndrome: successful treatment with carbamazepine. Br J Dermatol 1999;141:758-759. 4. Monrad SU, Terrell JE, Aronoff DM. The trigeminal trophic syndrome: an unusual cause of nasal ulceration. J Am Acad Dermatol 2004;50:949-952. 5. Weintraub E, Soltani K, Hekmatpanah J, et al. Trigeminal trophic syndrome: a case and review. J Am Acad Dermatol 1982;6:52-57. 6. Sadeghi P, Papay FA, Vidimos AT. Trigeminal trophic syndrome: report of four cases and review of the literature. Dermatol Surg 2004;30:807-812. 7. Koblenzer CS. Neurotic excoriations and dermatitis artefacta. Dermatol Clin 1996;14:447-455. 8. Koblenzer CS. Dermatitis artefacta: clinical features and approaches to treatment. Am J Clin Dermatol 2000;1:47-55. 9. 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. 10. Bull RH, Marsden RA, Cook MG, et al. Fatal facial ulceration. Clin Exp Dermatol 1993;18:356-359. 11. Howell RG. Trophic changes in the skin after operations on the trigeminal nerve. Br J Dermatol 1955;67:444-447. 12. Cohen cohen or kohen (Hebrew: “priest”) Jewish priest descended from Zadok (a descendant of Aaron), priest at the First Temple of Jerusalem. The biblical priesthood was hereditary and male. PR, Prystowsky JH. Traumatic and neurotropic neurotropic pertaining to or emanating from neurotrophy, e.g. neurotropic osteopathy. ulcers. In: Sams WM, Lynch PJ, eds. Principles and Practice of Dermatology. New York, NY, Churchill Livingstone, 1990. 13. Elloumi-Jellouli A, Ben Ammar S, Fenniche S, et al. Trigeminal trophic syndrome: a report of two cases with review of literature. Dermatol Online J 2003;9:26. 14. McKenzie KG. Observations on the results of the operative treatment of trigeminal neuralgia. CMAJ CMAJ Canadian Medical Association Journal 1933;29:492-496. 15. Bauer F. Trophic ulceration of the ala nasi following trigeminal denervation. J Laryngol Otol 1966;80:422-425. 16. Howell JB. Neurotrophic changes in the trigeminal territory: disturbances after operation for trigeminal neuralgia. Arch Dennatol 1962;86:442-449. 17. Abyholm FE, Eskeland G. Defect of the ala nasi following trigeminal denervation: case report. Scand J Plast Reconstr Surg 1977;11:87-90. 18. Ziccardi VB, Rosenthal MS, Ochs MW. Trigeminal trophic syndrome: a case of maxillofacial maxillofacial /max·il·lo·fa·cial/ (-fa´sh'l) pertaining to the maxilla and the face. max·il·lo·fa·cial adj. Relating to or involving the maxilla and the face. self-mutilation. J Oral Maxillofac Surg 1996;54:347-350. 19. Harper N. Trigeminal trophic syndrome. Postgrad Med J 1985;61:449-451. 20. Lyon CC, Mughal MZ, Muston HL. Herpetic trigeminal trophic syndrome in an infant. J R Soc Med 2001;94:135-137. 21. Dicken CH. Trigeminal trophic syndrome. Mayo Clin Proc 1997;72:543-545. 22. Mayer RD, Smith NP. Improvement of trigeminal neurotrophic ulceration with pimozide in a cognitively impaired elderly woman: a case report. Clin Exp Dermatol 1993;18:171-173. 23. McLean NR, Watson AC. Reconstruction of a defect of the ala nasi following trigeminal anaesthesia anaesthesia anesthesia. with an innervated forehead flap. Br J Plast Surg 1982;35:201-203. 24. Westerhof W, Bos JD. Trigeminal trophic syndrome: a successful treatment with transcutaneous electrical stimulation. Br J Dermatol 1983;108:601-604. Hedy G. Setyadi, BS, Philip R. Cohen, MD, Keith E. Schulze, MD, Stephen H. Mason, MD, Paul T. Martinelli, MD, Eugene L. Alford, MD, George E. Taffet, MD, and Bruce R. Nelson, MD From Baylor College of Medicine Baylor College of Medicine is a private medical school located in Houston, Texas, USA on the grounds of the Texas Medical Center. It has been consistently rated the top medical school in Texas and among the best in the United States. ; the Dermatologic Surgery Center of Houston, P.A.; the Department of Dermatology, University of Texas-Houston Medical School; and Alford ENT ENT ears, nose, and throat (otorhinolaryngology). ENT abbr. ear, nose, and throat ENT ear, nose and throat. ENT Ears, nose & throat; formally, otorhinolaryngology and Facial Plastic Surgery, Houston, TX. Reprint requests to Bruce R. Nelson, MD, Dermatologic Surgery Center of Houston, P.A., 6655 Travis, Suite 840, Houston, TX 77030. Email: Bnelson@dermsurgeryhouston.com Accepted August 1, 2006. RELATED ARTICLE: Key Points * Trigeminal trophic syndrome is an uncommon cause of chronic ulcers which can present as a self-induced nasal ulceration. * The pathogenesis of trigeminal trophic syndrome involves self-manipulation of the skin following either stroke, sensory denervation of the trigeminal nerve, or therapy for trigeminal neuralgia. * The differential diagnosis of nasal ulcers includes not only trigeminal trophic syndrome, but also factitial disorders, granulomatous conditions, infectious diseases, malignancy, and pyoderma gangrenosum. * A biopsy of a nasal ulcer is helpful to exclude other conditions in the clinical differential diagnosis of trigeminal trophic syndrome. * Treatment of trigeminal trophic syndrome requires prevention of digital manipulation of the lesion--either by occluding contact with the ulcer, initiating psychotropic medication, or both.
Table 1. Differential diagnosis of nasal ulcers
Factitial disorders with ulcerations (1-8)
Dermatitis artefacta
Neurotic excoriations
Granulomatous conditions (1,6)
Sarcoidosis (1)
Wegener granulomatosis (1,2,6,9,10)
Infectious diseases (6)
Anthrax (cutaneous) (4)
Aspergillosis (4)
Blastomycosis (1,4-6)
Herpes simplex infection (1,4)
Leishmaniasis (cutaneous) (1,2,4,6)
Leprosy (leprous trigeminal neuritis) (1,2,4-6)
Mucormycosis (4)
Paracoccidiomycosis (1,4-6)
Sporotrichosis (4)
Staphylococcus aureus infection (1)
Syphilis (1,2,4-6)
Tuberculosis (4,6)
Varicella zoster infection (2,4)
Yaws (4,5)
Malignancy (6,11)
Basal cell carcinoma (1,2)
Lymphoma (1,2,4-6)*
Sarcoma (6)
Squamous cell carcinoma (1,2)
Pyoderma gangrenosum (1,4-6)
Trigeminal trophic syndrome (1,4-6)
*This includes sinonasal (angiocentric) T/NK cell lymphoma[1,2,4] and
other conditions--such as lethal midline granuloma syndrome[1,5,6] and
polymorphic reticulosis[1]--which have more recently been reclassified
as lymphomas.
Table 2. Characteristics of self-induced ulcers: trigeminal trophic
syndrome versus factitial disorders
Characteristics Trigeminal trophic syndrome
Onset age More common in the elderly, (4,12) with a mean of 60
years of age. (6) Time of onset from trigeminal nerve
injury to ulceration varies from 2 weeks to 30
years. (6)
Sex More common in women. (4,12) Female to male ratio of up
to 2.2:1. (5)
Etiology Self-manipulation of the skin that most commonly
develops following therapy for trigeminal neuralgia,
which disrupts the Gasserian
ganglion. (4-6,15,16,18,19) Such therapies include
surgical trigeminal ablation by rhizotomy of the
dorsal trigeminal nerve root or alcohol
injection). (1,2,4-6,11,12,14,16) Another common
cause is stroke. (1,4,6,12,13) Other less common
causes include mental impairment (such as
dementia), (19) intracranial neoplasms, (2,4,5)
intracranial surgery, (14) trauma, (12) varicella
infection, (2) postencephalitic Parkinsonism,
syringobulbia, (5,6,11) craniotomy, (6) herpes
simplex infection (2,4,5,20) or mycobacterial
infection. (2,4)
Location Predominantly nasal ala and paranasal
areas. (2,5,6,12,13,14) The upper lip, (5,6,11)
cheek, (2,5,6) temple, (2) scalp, (2,5,6) forehead,
ear, palate, or jaw (4-6) may also be affected.
Typically unilateral. (6)
Appearance Crusted, (2,5,6,11,13) painless (2,5,6,13) crescent-
shaped (1,5,6,11) ulceration of the lateral nasal
ala. (1,4)
Other clinical Patients usually complain of ipsilateral facial
features paresthesia and/or trigeminal
anesthesia. (1,2,4,5,11-13,15) Most patients
voluntarily (2,3,6) or eventually (4) admit painless
self-manipulation. (5) May be preceded or accompanied
by ipsilateral nasal congestion, (1,5)
burning, (1,2,4-6,12,15) pruritus, crawling
sensation, (1,2,5,6,12,15) hearing loss, and/or
absence of corneal reflex. (2,11,13) Associated with
ocular lesions such as neurotrophic keratitis,
iritis, and corneal ulceration and
opacification. (1,4)
Histology Ulceration, (2,4) acanthosis of the epidermis, (2)
dermal inflammatory changes, (4,11) reactive vascular
and connective tissue changes in the dermis. (13)
Treatment Initially, provide local wound care, including topical
and systemic antibiotics if necessary. (1,2,4-6,21)
Occlusive dressings, (2) finger bandages, protective
gloves, nasal casts or prostheses to prevent digital
manipulation of the lesion. (1,4-6,21) Some emphasize
the importance of educating the patient about the
self-indued nature of the lesion. (4-6)
Medical Psychotropic medications such as carbamazepine 100 mg
treatment twice daily, (2-4,6) or diazepam, amitriptyline
hydrochloride, chlorpromazine, (2-4,6) clonazepam (4)
or pimozide. (22) Vitamin B supplementation has also
shown some success. (4-6)
Surgical High recurrence rate. (1,5,12) Only for lesions that
treatment have healed with conservative wound care and after
digital manipulation has ceased. (1) Contralateral
innervated forehead flaps (17,23) to prevent self-
tampering, ipsilateral cevical
sympathectomy, (4,11,12) or transcutaneous electrical
stimulation (2,4,6,24) have had some success.
Factitial disorders
Characteristics Dermatitis artefacta Neurotic excoriations (7)
Onset age Most common in late teens Mostly in the third to
or early twenties. (7,8) fifth decade.
Sex Predominantly women. Female Occurs more often in
to male ratio from 3:1 to women.
20:1. (7,8)
Etiology Consciously or unconsciously Compulsively self-
produced trauma from induced, the patient is
persistent self- usually conscious of
injury. (1,7,8) Skin creating skin lesions
lesions are created to but unable to stop. May
satisfy an internal start as a minor skin
emotional need that the problem such as insect
patient is not consciously bite or acne, and may
aware of. May be triggered also be triggered by
by significant psychosocial stressors.
psychological
stressors. (7,8) Most have
borderline personality
disorder. (2,3,7,8)
Location Areas within reach of hands, Extensor surfaces of
such as face, upper trunk, upper and lower
or extremities. extremities, face, and
upper trunk.
Appearance Lesions may be In advanced stages,
bilateral, (4) without lesions are in all
characteristics of a known stages of evolution,
dermatosis. Margins are including excoriations,
sharp, borders are circular or linear
angulated and geometric. lesions with
Can be crusted, necrotic, hypertrophic borders,
abraded, or atrophic. (8) to hypopigmented
atrophic scars.
Other clinical Normal trigeminal nerve Patient becomes caught in
features sensation (1) and usually an itch-scratch cycle.
denial of producing the Psychiatric evaluation
lesions. (1,7,8) Lesions can range from normal
are usually tender. (4) to severely ill; most
No history of neurologic commonly associated
insult. (6) Patient with an obsessive-
usually cannot elicit compulsive trait.
evidence of evolutionary Associated with
changes ("hollow history") generalized anxiety
and is usually ambivalent disorder or depression
about the lesions, in some. More likely to
although the lesions admit self-injury.
should be painful. Most
have personality
disorders, most commonly
with borderline
features. (8) Patient or
family member may have a
connection with healthcare
field, or have had serious
illness in
childhood. (7,8)
Histology Nonspecific and depends on Nonspecific inflammatory
how patient creates the changes.
lesion. (7)
Treatment Continue a supportive and Symptomatic, including
cautious relationship with topical antipruritics
patient to cultivate and such as 5% doxepin
maintain trust. Also refer cream, oral
patient to a mental health antihistamines,
professional. Confronting emollients with
the patient may result in menthol, cool
further denial and compresses, and topical
cessation of care. (1,8) antibiotics if
Nasal prosthesis is necessary. Refer to a
recommended for cosmetic mental health
purposes. (1) Baths, professional if
emollients, compresses and possible, for a
gentle debridement to combination of
allow nondestructive psychotherapy and
tactile gratification, and pharmacotherapy.
topical antibacterials if
needed. Oclusive dressings
may also be used. (8)
Medical Higher dose of serotonin- Serotonin-specific
treatment specific reuptake reuptake inhibitor to
inhibitor or low dose reduce compulsion and
atypical antipsychotics address the depressive
such as risperidone, component.
olanzapine, or
pimozide. (8)
Surgical Not recommended since Not recommended.
treatment condition is likely to
recur. If surgical
intervention is necessary,
wait until the lesion has
epithelialized, and
patient has ceased
manipulating the lesion
for a period of time. Also
consider having patient
cleared for surgery by a
mental health
professional. (1)
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