Symptomatic perineural and intraneural invasion of the trigeminal nerve and subsequent abducens Nerve palsy by cutaneous squamous cell carcinoma.

A 74-year-old woman who presented initially with trigeminal neuralgia of the left forehead and scalp was later found to have a poorly differentiated squamous cell carcinoma (SCC) with large-nerve perineural and intraneural invasion of the left supraorbital nerve. Negative histopathologic margins were achieved in three stages of permanent fixed tissue en face processing and the final defect was repaired with a large rotation flap. Approximately one month after repair, the patient presented with new-onset diplopia and was found to have a complete left cranial nerve VI palsy suspicious for continued disease spread. MRI confirmed perineural spread along the ophthalmic branch of the trigeminal nerve through the superior orbital fissure into the cavernous sinus. She was subsequently treated with radiation therapy (66Gy in 33 fractions). The involvement of two distinct cranial nerves by perineural invasion is uncommon and has mostly been described involving branches of the trigeminal and facial nerves. This case highlights the rare presentation of perineural invasion involving both the trigeminal nerve and the abducens nerve. Anatomically, this clinical presentation can be explained by the retrograde perineural spread along the ophthalmic branch of the trigeminal nerve through the supraorbital fossa into the cavernous sinus where these two nerves are in close proximity.

Toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in pediatric patients with a focus on newer antiepileptic drugs: A 25-year retrospective study at a single tertiary care center.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis Syndrome (TEN) are rare immune-mediated diseases. Extensive research on adult triggers of SJS and TEN is available; however, research in children is more limited. OBJECTIVE: We sought to investigate and report the experience with pediatric SJS and TEN in our center, identifying associated medications. METHODS: A retrospective review from 1990 to 2015 at the Shriner's Burn Hospital in Galveston, Texas was performed to identify patients diagnosed with SJS, SJS/TEN overlap, and TEN. Data pertaining to demographic characteristics, medical history, physical exam, treatment, and outcomes were collected. RESULTS: We identified SJS/TEN overlap or TEN in 51 patients. Antiepileptic drugs were the most common group of causative agents, closely followed by antibiotics. The most common causative agents were trimethoprim-sulfamethoxazole, phenytoin, and lamotrigine used concomitantly with valproic acid. Newer generation agents, with the definition of agents approved after 1990, were the cause in 13/51 (25.5%) cases. Newer generation agents included lamotrigine, clobazam, and zonisamide. Seven patients died, resulting in a 13.7% mortality rate. Renal failure, liver failure, sepsis, and gastrointestinal involvement each had a statistically significant association with mortality. SCORTEN was statistically significantly greater in patients who died compared to children who lived (3 vs 2). LIMITATIONS: This is a retrospective study. CONCLUSION: Three drugs introduced into the market since 1990 have emerged as causes of SJS/TEN overlap and TEN: lamotrigine, clobazam, and zonisamide. These medications are being used more widely to treat seizures, as well as mood disorders. It is also important for clinicians to be aware of the extremely commonly used medications such as amoxicillin, tetracyclines, NSAIDs, and acetaminophen that can rarely cause SJS and TEN.

Managing pain associated with dermatologic procedures.

Dermatologists are faced with an aging population, accompanied by an increase in the incidence of skin cancer, especially nonmelanoma skin cancer. With this increase in cutaneous malignancy comes an increase in the number of dermatologic procedures being performed. A common concern of dermatologists and dermatology patients is the attenuation and management of pain associated with dermatologic procedures. Fortunately, there are several techniques that may be used in the preoperative, intraoperative, and postoperative periods to manage patient anxiety, minimize injection pain, and safely and effectively treat postoperative pain.

Basal cell carcinoma masquerading as a dermoid cyst and bursitis of the knee.

Basal cell carcinoma (BCC) is the most common malignant skin cancer diagnosed in the United States. We present the case of a 67-year-old man whose knee pain and tumor of 2 years' duration was initially diagnosed as a dermoid cyst and bursitis. Dermatology referral confirmed a delayed diagnosis of BCC. This case provides insight into the range of BCC presentations and reminds clinicians to consider BCC when the differential diagnosis includes a dermoid cyst and bursitis.

Nephrogenic systemic fibrosis: A 15-year retrospective study at a single tertiary care center.

BACKGROUND: Despite multiple therapeutic approaches for nephrogenic systemic fibrosis (NSF), no single treatment has convincingly shown consistent benefit. The most successful outcomes have been associated with recovery of renal function, although evidence remains limited and past studies have been inconclusive. OBJECTIVE: We sought to investigate whether improvement of renal function via successful transplantation or via return of renal function after acute kidney injury correlates with improvement of NSF, and to further characterize the clinical features and progression of NSF. METHODS: A retrospective medical chart review led to the identification of patients (n = 8) diagnosed with NSF who presented to a single academic tertiary referral center over a 15-year period. These 8 patients were contacted by phone to obtain information related to treatment and clinical course of their NSF and renal function. Statistical analysis was performed using Fisher's exact test. RESULTS: There is a significant correlation (P = .0286) of improved renal function with improvement of NSF. All 4 patients who had improvement of renal function also had improvement of NSF. Two of these patients had end-stage renal disease and a successful kidney transplant, and two had acute kidney injury that resolved. No improvement in NSF was observed without kidney function resolution. LIMITATIONS: Our study is limited by a small sample size (n = 8) and a retrospective study design, which increased its potential for selection and recall bias. CONCLUSION: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.

Cutaneous Metastasis From Sacral Chordoma.

Chordoma is a rare primary bone malignancy of notochord origin, representing 1-4% of malignant bone tumors., Typically, chordomas follow a slow progressive course with aggressive local extension, multiple recurrences, and metastases. Of particular interest to this case, cutaneous metastasis is exceedingly rare. Diagnosis of this entity can be a challenge due to the rarity of chordoma, as well as the infrequent presentation of distant cutaneous metastasis and non-specific clinical skin findings. We report a case of a 61-year-old male with a history of sacral chordoma treated by wide local excision 8 years prior to presentation developed a nodule on his scalp for 6 weeks. Physical examination revealed a 1 cm rubbery, pink, shiny dome-shaped nodule on his left occipital scalp. Hematoxylin and eosin sections revealed a lobular dermal proliferation of small ovoid cells and larger physaliferous cells with hyperchromatic, displaced nuclei and finely vacuolated "soap-bubble" cytoplasm in a myxoid stroma. Immunohistochemistry of tumor cells showed positivity for both S-100 protein and pancytokeratin (AE1/AE3), while smooth muscle actin (SMA), P63, and CK7 were negative. Additionally, tumor cells stained positive for brachyury. The medical history, clinical presentation, histopathological appearance and immunohistochemical profile are consistent with cutaneous metastasis from sacral chordoma, known as chordoma cutis. This case illustrates the integral role of dermatopathology in the diagnosis of a rare and critical condition.