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62-Year-Old Male with Raised Facial Rash

Can you diagnose this case?

Jake Berriocha, MD, and David Effron, MD – Series Editor – 2016

Signs and Symptoms

A 62-year-old man presented with a facial rash. It started 3 days prior on the left cheek and progressed inferiorly and laterally along his left neck and upper chest. Initially, the rash was red and raised, but continued to form multiple blisters. There was an associated severe burning pain, and it was nonpruritic.

The man denied using any new facial creams or cleaning products. He had a recent history of esophageal cancer, for which he had completed chemotherapy and radiation therapy 4 weeks prior. His course had been complicated by a deep-vein thrombosis, prompting initiation of enoxaparin 1 week prior. He denied any fever, myalgia, arthralgia, nausea, or vomiting.

Physical Examination: On examination, the patient was in no acute distress, and his vital signs were normal. He had no scleral injection. Mucous membranes were moist, and there were no intraoral lesions, swelling, or asymmetry. There was no trismus or stridor, and the patient was able to manage his secretions without difficulty. There was a large swelling under his chin that was firm and only mildly tender. The area under his tongue was soft. No axillary adenopathy was appreciated.

Cardiovascular examination findings were normal, and the lungs were clear. There was no abdominal pain. No neurologic deficits were identified. Skin examination findings were notable for a rash on the left face, posterior to the left ear, left neck, and of the left-sided upper chest. The rash was composed of multiple vesicles and pustules of various sizes with erythematous bases. It did not cross the midline and was negative for the Nikolsky sign.

He received morphine to alleviate the pain while tests were conducted. A complete blood cell count showed no leukocytosis or anemia. The results of a basic metabolic panel were normal. The C-reactive protein level was notably elevated at 3.8 mg/L.

What’s your diagnosis?

Choose one to reveal diagnosis and discussion

Stevens-Johnson syndrome (SJS) / Toxic epidermal necrolysis (TEN)
Pemphigus vulgaris
Herpes zoster
Radiation dermatitis

Answer: Herpes zoster

See the full case at Consultant360

This patient had herpes zoster (shingles), an infection caused by the reactivation of latent varicella-zoster virus in the cranial nerve ganglia or dorsal root ganglia. The virus travels along the sensory nerves, causing a vesicular rash in a dermatomal distribution. Zoster classically affects a single dermatome, although as in this case it occasionally affects 2 or 3 neighboring dermatomes, which can make diagnosis more difficult.

A herpes zoster outbreak typically begins as macules and erythematous papules, which then evolve into vesicles and, ultimately, pustules. The rash often is preceded by 2 to 3 days of paresthesias, itching, or pain. The greatest risk factor is age, and unvaccinated persons who live to 85 years have a 50% risk of developing herpes zoster.

Antiviral therapy is the recommended treatment. These agents hasten the resolution of lesions and decrease the severity of pain but have not been shown to reduce the risk of postherpetic neuralgia.1

Other diagnoses to consider in this patient’s case are SJS and TEN, due to the large bullae and broad distribution of the rash. These conditions represent a spectrum of disease, are believed to be an immune-mediated response, and most commonly are associated with the use of anticonvulsants, sulfonamides and other antibiotics, and nonsteroidal anti-inflammatory drugs.2 They can both present initially with a prodromal viral-like 3

The rash of SJS typically starts on the trunk; conversely, TEN starts on the face and neck and progresses inferiorly. Mucous membrane involvement is common in both, and stomatitis or conjunctivitis is typical. The Nikolsky sign will be positive, and the degree of epidermal detachment is the distinguishing feature between SJS and TEN, with TEN affecting greater than 30% of body surface area. Treatment of both conditions is supportive, and the mortality rate is 5% to 10% for SJS and 25% to 30% for TEN.4

Also on the differential diagnosis list is pemphigus vulgaris, a rare autoimmune disease. It is characterized by epidermal erosions and blistering of the skin that typically affects the elderly. Initially, blistering of the oral mucosa precedes the skin lesions by weeks to months.5 The rash typically is localized and progresses down from the face and trunk.5 It is painful and nonpruritic, and the Nikolsky sign is positive. The mortality rate of pemphigus vulgaris is 10% to 20% when treated with corticosteroids.6

Our patient had been treated with radiation therapy for his esophageal cancer in the recent past, putting him at risk for radiation dermatitis. This condition features a range of mild to severe symptoms characterized by erythema, edema, bullae, and dry or moist desquamation in radiated locations other than skin folds.7 The onset typically is within days to weeks of initiating therapy, and reepithelialization usually begins within 10 days after radiation exposure in the absence of infection.8 Radiation dermatitis would not be limited to one side of the neck and would present more diffusely across the previously treated area.

Outcome of the Case:In the emergency department, the patient was given intravenous fluids, acyclovir, vancomycin, and piperacillin-tazobactam to cover for a secondary bacterial infection. A computed tomography scan of his neck was performed, looking for possible deep neck infection, although only soft tissue edema was found. The patient was admitted to the general medicine floor. The lesion was biopsied and cultured, and the results were consistent with herpes zoster.

The patient was treated with acyclovir and prednisone, and his lesions began to crust over. On hospital day 5, he was discharged home. A follow-up visit several weeks later demonstrated complete resolution of the patient’s lesions.

References:

1. Cohen JI. Clinical practice: herpes zoster. N Engl J Med. 2013;369(3):255-263.

2. Roujeau JC, Kelly JP, Naldi L, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med. 1995;333(24):1600-1607.

3. Nguyen T, Freedman J. Dermatologic emergencies: diagnosing and managing life-threatening rashes. Emerg Med Pract. 2002;4(9):1-28.

4. Revuz J, Penso D, Roujeau J-C, et al. Toxic epidermal necrolysis: clinical findings and prognosis factors in 87 patients. Arch Dermatol. 1987; 123(9):1160-1165.

5. Seidenbaum M, David M, Sandbank M. The course and prognosis of pemphigus: a review of 115 patients. Int J Dermatol. 1988;27(8):580-584.

6. Rosenberg FR, Sanders S, Nelson CT. Pemphigus: a 20-year review of 107 patients treated with corticosteroids. Arch Dermatol. 1976; 112(7):962-970.

7. Hymes SR, Strom EA, Fife C. Radiation dermatitis: clinical presentation, pathophysiology, and treatment 2006. J Am Acad Dermatol. 2006; 54(1):28-46.

8. McQuestion M. Evidence-based skin care management in radiation therapy: clinical update. Semin Oncol Nurs. 2011;27(2):e1-e17.

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