Ear, Nose & Throat Journal2023, Vol. 102(10) 629 –631© The Author(s) 2021Article reuse guidelines:sagepub.com/journals-permissionsDOI: 10.1177/01455613211022098journals.sagepub.com/home/ear
Acute febrile neutrophilic dermatosis (Sweet syndrome) is a rare idiopathic condition characterized by fever and whole-body rash of tender erythematous plaques of unknown etiology. Otorhinolaryngologic manifestations of the disease can be severe, yet they are sparsely reported in the literature. We present the first documented case of laryngeal involvement of Sweet syndrome.
A 62-year-old, previously healthy male presented to the emergency department at a tertiary medical center with complaints of worsening pharyngitis, headache, full-body rash, myalgias, and odynophagia with decreased oral intake. His symptoms began 8 days prior with pharyngitis and myalgias for which he was seen at an urgent care and prescribed antibiotics. On day 4 of symptoms, he developed sudden onset of a full-body rash and hoarseness without respiratory distress. The next day he noted severe restriction in his oral intake due to odynophagia. He was admitted to the hospital for dehydration and inability to tolerate significant PO. Otorlaryngology was consulted due to his oral complaints and sudden onset of hoarseness.
Computed tomography (CT) neck performed in the emergency department was negative for abscess, whereas CT chest demonstrated multifocal pneumonia. Infectious workup was negative, including viral respiratory panel, rapid strep test, mononucleosis, enterovirus polymerase chain reaction (PCR), herpes virus PCR, syphilis, HIV, varicella zoster skin scraping, and histoplasmosis screen. On physical exam, he had multiple discrete red vesicular targetoid papules distributed evenly across the chest, abdomen, back, arms, and legs (Figure 1). ENT exam revealed a pink and moist oropharynx with t notable erythematous, raised lesions on the uvula, and right anterior tonsillar pillar, respectively. Flexible laryngoscopy demonstrated diffuse supraglottic edema with minor pooling of secretions. White papules were noted in the mucosa overlying the epiglottis descending into the glottic inlet (Figure 2).
Conservative management of his oropharyngeal symptoms was pursued utilizing magic mouthwash and analgesia. Skin biopsy was performed demonstrating focal spongiosis and parakeratosis with marked subepidermal edema and immune cell infiltration consistent with acute febrile neutrophilic dermatosis (Sweet syndrome). The patient was treated with high-dose steroids with improvement in his symptoms. He was referred for oncologic evaluation while inpatient which did not yield any abnormalities. He was discharged on postadmission day 4 after much improvement, able to tolerate oral intake.
Sweet syndrome is a rare cause of full-body rash and pharyngitis which can progress to clinically significant laryngitis.1-3 This is the first report of Sweet syndrome involving laryngeal structures in the literature. While most cases are idiopathic, it is also known to be associated with occult malignancy (most commonly acute myeloid leukemia and genitourinary tumors) and is known to be induced by medication changes (most commonly granulocyte stimulating factor, antibiotics, or immunotherapy; Table 1). It is important to refer patients for oncologic surveillance given the association with occult malignancy. Approximately 1/3 of patients with idiopathic disease will recur within their lifetimes.4 Treatment consists of antiinflammatory medications including high-dose steroid taper.5 Due to supraglottic edema and potential airway compromise, it is an important diagnosis to consider in patients presenting with odynophagia and diffuse dermatologic findings.
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Brian A. Walker https://orcid.org/0000-0002-5734-4889
Supplemental material for this article is available online.
1 Department of Otorhinolaryngology, The Mayo Clinic Arizona, Phoenix, AZ, USA
Received: May 07, 2021; accepted: May 15, 2021
Corresponding Author:Brian A. Walker, MD, Department of Otorhinolaryngology, The Mayo Clinic Arizona, 5777 E Mayo Blvd, Phoenix, AZ 85054, USA.Email: walker.brian@mayo.edu
