Case reports: unilateral facial paralysis after treatment of secondary syphilis

Journal of Drugs in Dermatology, June, 2008 by Emily M. Berger, Hassan I. Galadari, Alice B. Gottlieb

Abstract

Bell's palsy is an acute facial paralysis of unknown etiology. Infections including syphilis have been implicated as causes for peripheral facial paresis. The Jariseh-Herxheimer reaction is an acute worsening of skin manifestations and systemic symptoms occurring after administration of antimicrobial therapy for spirochetal infections. Although rare, neurological signs can present as part of the Jariseh-Herxheimer reaction. The authors report a case of Bell's palsy experienced by a patient shortly after treatment with penicillin for secondary syphilis and propose that this acute unilateral peripheral facial paralysis was a Jariseh-Herxheimer reaction in response to therapy.

Introduction

Most cases of unilateral peripheral facial weakness or paralysis have an unidentified etiology and are referred to as Bell's palsy. (1), (2) Bell's palsy takes its name from Sir Charles Bell, a Scottish anatomist and surgeon who described the phenomenon in the early 19th century, although earlier accounts of idiopathic facial paralysis dating as far back as the early 17th century exist. (3) Patients with Bell's palsy have decreased facial movement or are not able to move the facial muscles on one side of the face. (4) They may have accompanying symptoms such as hyperacusis, decreased tear production, altered taste on the anterior tongue, and mild pain. (1), (4) Even untreated, the majority of patients experience complete recovery within several weeks. (1) A delayed improvement in facial paralysis is a poor prognostic sign that indicates a patient may not regain complete nerve functional Although the exact etiology and pathogenesis of Bell's palsy is unknown, it is likely the result of a herpes virus reactivation in at least one third of cases with subsequent facial nerve inflammation. (1), (4), (5) In some patients, nerve inflammation may follow an acute exposure to wind or cold or it may follow soon after an upper respiratory infection in an otherwise well person. (6)

Acute unilateral facial weakness or paralysis with an identified cause is not considered Bell's palsy. Cases of unilateral facial paralysis have been reported in patients in the second or third stage of syphilis with syphilitic meningitis. (7), (9) Other identified central or peripheral causes include stroke, trauma, tumor, middle ear disease, Lyme disease, diabetes, hypertension, human immunodeficiency virus infection, sarcoidosis, Sjogren's syndrome, and Ramsay Hunt Syndrome involving varicella zoster virus infection at the geniculate ganglion. (1), (2)

The authors describe a case of unilateral facial paralysis that began shortly after the administration of antimicrobial treatment for secondary syphilis in a young man. This case broadens the relationship between facial palsy and syphilis. Acute unilateral facial paralysis can be a novel form of the Jariseh-Herxheimer reaction associated with antimicrobial treatment of Treponema pallidum infection.

Case Report

A 19-year-old African American male presented with a pruritic eruption on the face, chest, back, upper extremities and abdomen that began 2 weeks prior to his presentation. The patient reported an accompanying patchy alopecia and partial loss of eyebrow hair. He was otherwise well and denied nausea, vomiting, fever, myalgia, joint pains, respiratory distress, or headache. The patient denied the use of medications or recreational drugs. He was hesitant in disclosing a recent unprotected homosexual encounter and denied any genital or perianal lesions. He had no family history of connective tissue disease or sarcoidosis. Physical examination revealed multiple erythematous papules and plaques on the face, chest, abdomen, back, and upper extremities. The lesions on the face were indurated (Figure 1). In contrast, lesions elsewhere on the body were hyperkeratotic and scaling (Figure 2). The eruption spared the palms, soles, and the periorbital area. There were no identifiable lesions on the genitals or buttocks. On the scalp, there was subtle evidence of a non-scarring, patchy alopecia (Figure 3).

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Blood tests revealed an elevated white blood cell count and platelet count. In addition, the erythrocyte sedimentation rate was elevated at 112 (reference range 0-15 mm/hr). Both the rapid plasma reagin (titer of 1:2.56) and Treponema Pallidum Particle Agglutination were reactive. Antinuclear antibody and human immunodeficiency virus screens were negative.

A biopsy taken at the time of presentation revealed a lichenoid infiltrate consisting of epithelioid and giant cells admixed with plasma cells and neutrophils. Clinical, laboratory, and pathological evidence thus supported the diagnosis of secondary syphilis.

The patient was treated with penicillin G 2.4 million units by intramuscular injection. Two days after treatment the patient presented to the local emergency department complaining of a right peripheral facial paralysis with associated mild headache and fatigue, but he denied photophobia, neck stiffness, fever, and upper respiratory symptoms. The patient noted that his rash had not improved by that time, and it continued to he very pruritic. He denied noticing any new lesions on the mucous membranes or skin including the mouth, lips, genitals, or other areas of the body. Physical examination revealed vital signs within normal limits. Neurological examination revealed normal sense of smell and hearing, intact visual fields, and intact extraocular movements. The patient's pupils were equal, round, and reactive to light and accommodation. The corneal reflex and facial sensation were normal. Cranial nerves IX through XII were similarly intact. An examination of cranial nerve VII revealed right-sided facial droop and facial asymmetry with loss of forehead wrinkling on the right side. The patient was unable to shut the right eye tightly, grin, or purse his lips to whistle. Physical examination of the skin was unchanged from his original presentation. There were no mucosal or genital lesions. The patient was HIV negative, his complete blood count was within normal limits, and the rapid plasma reagin was reactive. Imaging and electromyography studies were deemed unnecessary at that time.