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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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Author:Nelson, Bruce R.
Publication:Southern Medical Journal
Article Type:Disease/Disorder overview
Geographic Code:1USA
Date:Jan 1, 2007
Words:3862
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