Trigeminal trophic syndrome mimicking squamous cell carcinoma
Yasir Al-Dojaily, BMSc (aldojaily@vcu.edu)1, Jessica Payne, MD (Jessica.payne@vcuhealth.org)2, Jennifer Crimmins, MD (Jennifer.Crimmins@vcuhealth.org)2, and Shields Callahan, MD (shields.w.callahan@vcuhealth.org)2
1Virginia Commonwealth University School of Medicine, Richmond, Virginia; 2Department of Dermatology, Virginia Commonwealth University Health System, Richmond, Virginia
Key words: trigeminal trophic syndrome; dysesthesia; skin ulcer; squamous cell carcinoma; facial dermatoses; stroke complications
Corresponding author: Yasir Al-Dojaily, 208-8 East Leigh St, Richmond, VA 23219, 804-496-0757
ORCID 0000-0003-4735-0908
Manuscript word count: 760
Reference count: 10
Figure count: 2
Funding statement: This article has no funding source.
Conflicts of interest statement: The authors have no potential conflicts of interest to declare.
The authors confirm that this case report has not been presented elsewhere.
Reprint request to: Corresponding Author
Written informed consent was obtained from the patient for the publication of photographs and medical information in print and online, with the understanding that this information may be publicly available.
INTRODUCTION
Trigeminal trophic syndrome (TTS) is a rare cause of persistent facial ulceration. Lesions arise from repetitive self-inflicted skin trauma after insult to the trigeminal sensory system.1 Facial dysesthesia and anesthesia result from damage to the trigeminal nerve or sensory nuclei and provoke excessive skin-picking.2Clinically, TTS resembles other primary cutaneous diseases and is often misdiagnosed due to its rarity.3,4 Thorough neurologic history and examination are essential to accurately diagnose the disorder. Herein we present a unique case of TTS clinically and histologically mimicking cutaneous squamous cell carcinoma (SCC).
CASE REPORT
A 56-year-old man with history notable for recent ischemic stroke presented to an outpatient dermatology clinic for evaluation of progressive ulcerations on the face that initially started on the right nasal ala and spread to involve the right nasolabial fold and right upper and lower cutaneous lip (Figure 1). The patient reportedly had a nevus on the right side of his nose, which he picked off prior to symptom onset. He was treated with multiple courses of antibiotics for suspected soft tissue infection but his symptoms persisted and he developed progressive tissue loss and retraction over the right alar rim. Multiple tangential biopsies were obtained and revealed atypical squamous proliferation. The patient was referred for Mohs micrographic surgery for a presumed diagnosis of well-differentiated SCC.
Punch biopsies of the lesions at 3-mm thickness were performed to confirm the diagnosis and determine depth of involvement. Repeat histopathology demonstrated findings consistent with lichen simplex chronicus/prurigo-like changes including hyperkeratosis, patchy parakeratosis, marked irregular acanthosis, and hypergranulosis. Focal ulceration was also seen. The dermis showed fibrosis and chronic inflammation (Figure 2). No significant keratinocyte atypia was observed. Given the history of self-manipulation and likely neurologic mechanism involving trigeminal anesthesia in the setting of a recent ischemic stroke, a final diagnosis of TTS was made. The patient was educated on the self-inflicted cause of the ulcers and advised to avoid manipulating the sites.
DISCUSSION
TTS is a rare cutaneous dysesthesia that results in chronic facial ulceration due to repeated self-mutilation and excoriation of the skin. The syndrome is most frequently reported as an iatrogenic consequence of trigeminal ablation for trigeminal neuralgia.1 Less common causes include tumors, herpes zoster, Hansen disease, and posterior circulation stroke, as seen in our patient. Treatment consists primarily of behavioural modification to reduce skin trauma. Medications such as carbamazepine and amitriptyline can reportedly reduce paresthesia to prevent further skin manipulation.1,5
Diagnosis of TTS relies on thorough neurologic history and physical examination to reveal evidence of damage to the trigeminal nerves or its nuclei. Paresthesia and anesthesia ipsilateral to the skin lesion are classic in TTS, although bilateral TTS has been reported.6 Paresthesia promotes skin-picking to relieve abnormal cutaneous sensations while anesthesia permits prolonged painless skin trauma.2 Our patient demonstrated an atypical mechanism as he lacked a cutaneous paresthesia trigger and instead self-manipulated due to the visual perception of skin blemishes. He also reported experiencing a sensation of nasopharyngeal drainage, which has been previously described in association with trigeminal anesthesia and may serve as a diagnostic clue to TTS.1
TTS may resemble neoplasms, granulomatous diseases, and various infections including leprosy, cutaneous tuberculosis, and primary syphilis. Patients are often misdiagnosed and delayed care because the condition’s rarity and mimicry allow it to elude the differential diagnosis for persistent skin ulceration.3,7 Lesions tend to present as well-demarcated, sickle-shaped ulcers, most frequently around the nasal ala.1 Histopathology can help differentiate TTS from other primary cutaneous disorders and is expected to show nonspecific inflammatory changes consistent with chronic mechanical trauma, including dermal fibrosis, hyperkeratosis, hypergranulosis, and lichenification.1,2,7 As demonstrated in our case, TTS can be histologically identical to nodular prurigo, another secondary skin disorder caused by chronic rubbing.
Superficial biopsies of our patient’s lesions initially demonstrated atypical squamous proliferation that led to early suspicion for well-differentiated cutaneous SCC. While TTS has been known to clinically resemble SCC, there are no previous reports of this syndrome containing histologic features of SCC.8,9 Atypical squamous proliferation is most commonly associated with cutaneous SCC, however it can also appear in actinic keratosis, warts, and benign inflammatory dermatoses like prurigo nodularis.10 As a nonspecific histologic description, the finding of atypical squamous proliferation alone likely indicates an insufficient or shallow sample, and it would be responsible practice to obtain a deeper repeat biopsy if possible to confirm the diagnosis. Our unique case confirms that atypical keratinocyte proliferation may also present in TTS, likely due to chronic inflammation caused by repeated skin-manipulation. Dermatologists should be aware of this unusual histopathological manifestation of TTS to avoid misdiagnosis, unnecessary surgery, and delay in treatment.
ABBREVIATIONS AND ACRONYMS
TTS: trigeminal trophic syndrome
SCC: squamous cell carcinoma
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Figure 1. Unilateral erosions, ulcerations, and alar rim retraction upon initial presentation (A) and at subsequent visit (B).