A Sweet Voice: Acute Febrile Neutrophilic Dermatosis of the Larynx

Abstract

Significance Statement

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.

Case Report

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).

Figure 1.

Full-body rash.

Figure 2.

Laryngeal lesions, (A) epiglottic involvement, and (B) glottic involvement.

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 anti-inflammatory 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.

Table 1.

Diagnostic Criteria of Acute Febrile Neutrophilic Dermatosis (Adapted from Cohen et al ¹ ).

Diagnostic criteria of Sweet syndrome (acute febrile neutrophilic dermatosis) 1
Classic Sweet syndromea
Major Criteria
1. Abrupt onset of tender or painful erythematous or violaceous plaques or nodules Predominantly neutrophilic infiltration in dermis without leukocytoclastic vasculitis
Minor criteria
Pyrexia >38 Celsius Association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, OR preceded by a URI, GI infection, or vaccine Good response to systemically administered corticosteroids and not to antibiotics Increased ESR, CRP, or CBC with >70% neutrophils
Drug-induced Sweet syndromeb
Abrupt onset of tender or painful erythematous or violaceous plaques or nodules Predominantly neutrophilic infiltration in dermis without leukocytoclastic vasculitis Pyrexia >38 Celsius Temporal relation between use of medication and clinical presentation or relapse with administration Disappearance of lesions after drug discontinuation or treatment with systemic corticosteroids

^a Classical Sweet syndrome: 2/2 major criteria, 2/4 minor criteria. Malignancy-associated Sweet syndrome: classic criteria alongside diagnosed malignancy.

^b Drug-induced Sweet syndrome: All 5 criteria must be present (adapted from Cohen ¹ )

Supplemental Material

Supplemental Material, sj-pdf-1-ear-10.1177_01455613211022098 - A Sweet Voice: Acute Febrile Neutrophilic Dermatosis of the Larynx

Supplemental Material, sj-pdf-1-ear-10.1177_01455613211022098 for A Sweet Voice: Acute Febrile Neutrophilic Dermatosis of the Larynx by Brian A. Walker, Lindsey B. Stull and J. Peyton Hines in Ear, Nose & Throat Journal