A rare case of nasal gout

  1. Emma Richards ,
  2. Emma Watts and
  3. Lisha McClelland
  1. ENT, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  1. Correspondence to Emma Richards; emma.richards13@nhs.net

Publication history

Accepted:18 Sep 2020
First published:30 Oct 2020
Online issue publication:30 Oct 2020

Case reports

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Abstract

Gout is an increasingly common metabolic disorder worldwide. Classical presentation is with acute attacks of arthritis affecting the first metatarsophalangeal joint. With disease progression, tophi may also appear. We present an unusual case of nasal gout in a 55-year-old man who was referred to the Ear, Nose and Throat department with irregularity over the nasal bones and episodic pain. We discuss the work up, diagnosis and management of this case and review the limited literature on this topic.

Background

Gout is a well-known metabolic disorder associated with joint inflammation. It is already the most common inflammatory arthritis worldwide and continues to increase in prevalence.1 The disease classically affects men (M:F ratio 4.3:1) and is rare in those under 20 years, increasing in prevalence until the ninth decade.2 Other risk factors include genetic factors and certain medications such as diuretics.3

Pathophysiologically, gout is a disorder of purine metabolism leading to hyperuricaemia as a sequelae of impaired renal excretion of urate. The disease trajectory is categorised into three parts: asymptomatic hyperuricaemia, periods of acute attacks of gouty arthritis and a final stage of chronic tophaceous gout.3 4 During acute attacks, symptoms of severe pain, swelling and erythema occur, usually becoming most intense in the first 24 hours. Following recurrent acute attacks, around 50% of people with untreated gout will develop tophi by 10 years.5 Clinically, gout typically manifests in the extremities because the lower temperature is more condusive to the precipitation of urate from the plasma.5 In roughly 75% of cases, the first metatarsophalangeal joint is affected first.3 Other areas commonly affected include the tarsals, ankles, knees and finger joints.3

Negatively birefringent urate crystals seen on polarising microscopic examination of specimens is the gold standard for diagnosis. However this is often impractical. Serum uric acid levels and joint X-rays also have a role,6 but in most cases it is the history and examination that generates the highest suspicion. Our case emphasises the importance of careful history and examination in the establishment of a strong differential when presented with an unusual manifestation of this condition.

Case presentation

A 55-year-old man presented to his General Practice with a 4-year history of intermittent swelling and redness over the bridge of his nose following a 35-year history of gouty arthritis. These episodes were associated with nasal obstruction and correlated with his joint pains, which were usually managed with diclofenac (having previously been intolerant of colchicine). He had no other significant medical history, but he was becoming conscious of the cosmetic impact of his nasal changes, particularly at his work place. On examination, he was found to have a tophus of his left elbow.

His GP commenced him on allopurinol and referred him to rheumatology for further opinion. Rheumatology review corroborated the history and examination and an ultrasound of his nose and CT scan of the sinuses were ordered. Referral was made to the Ear, Nose and Thorat (ENT) for further management.

ENT review confirmed the obvious irregularity overlying the nasal bones with increased pigmentation of overlying skin (figure 1). The episodes of swelling, redness and tenderness were associated with fluctuations in smell and nasal obstructive symptoms, therefore a nasendoscopy was performed to rule out underlying masses.

Figure 1

Examination revealed irregularity and increased pigmentation over dorsum of nose.

Investigations

  • Ultrasound of the nose: suggested non-specific diffuse swelling across the nasal bridge.

  • Flexible nasendoscopy: ruled out any underlying mass.

  • CT scan of the sinuses (figure 2): demonstrated a subcutaneous lesion around the dorsum of the nose with flecks of calcification, which could be consistent with gouty deposits.

Figure 2

CT scan of the sinuses shows flecks of calcification over the dorsum of the nose.

Differential diagnosis

The initial differential diagnosis included tophaceous gout due to the coincidence of nasal symptoms with flares of known gout affecting the metatarsophalangeal joints and elbows. Other differentials included soft tissue and bony neoplasms

Treatment

Following informed discussion, the patient opted to undergo debulking biopsy using an intercartilaginous approach.

Clinically, the patient benefitted from a good cosmetic outcome. Histology confirmed deposits of amorphous material interspersed within fibroadipose tissue and skeletal muscle fibres. Needle-like clefts were associated with a foreign body type granulomatous reaction and patchy dense chronic inflammation, supporting a diagnosis of gout.

Outcome and follow-up

Following biopsy and diagnosis, he was referred back to the rheumatology team for ongoing management.

Discussion

As the incidence of gout increases, so too does the variability of its clinical presentation, which extends beyond the characteristic joint arthropathy that is classically reported. The literature reports tophaceous lesions presenting in the breast, ears, spine, abdominal striae and even heart valves.7 Nasal tophi, however, remain a rare manifestation with fewer than 10 reported cases in the English language. This presentation fits with the tendency for crystals to precipitate in cool extremities.

All reported cases feature middle-aged males presenting with predominant symptoms of nasal obstruction and poor cosmetic appearance that improved following surgical management.8 As in this case, the nasal lesion is frequently tender, however a number of patients have reported painless lumps.9 10

All reported cases (bar one) have occurred in patients with known gouty arthritis and often poorly controlled serum urate levels.11 This reflects the natural progression of the condition as tophi typically develop during later stages. Thus, nasal tophi are unlikely to be the initial presentation of this systemic condition. Although this case demonstrated no features of bony invasion, Wu et al describe a 2×2×1.5 cm lesion on the nasal dorsum associated with nasal bone destruction which required repair with a titanium mesh plate.8 Similarly, CT imaging provided by Chen et al revealed a tophaceous lesion invading into the nasal bone and septal cartilage.10

CT scan proved the investigation of choice across all available literature reports. The majority of published cases were managed surgically and CT scan helped in operative planning. Chen et al advocated the role of ultrasound guided fine needle aspiration cytology (FNAC) and microscopy for diagnostics. This non-invasive option was particularly beneficial as their patient declined surgical management, electing to optimise medical therapy once reassured of the nature of the lesion.10 Papanicolaou (PAP) staining and polarised light microscopy may have a role in confirming pathology prior to surgical excision.10 Ultrasound has proven a useful investigation of tophi elsewhere in the body and is cost-effective, non-invasive and easy to perform.12 However, in our case, where no FNAC was performed, ultrasound provided little diagnostic assistance. Ultrasound FNAC therefore should be considered a safe and quick way to establish a diagnosis prior to definitive management. Flexible nasendoscopy is another relevant investigation, which to our knowledge was only explicitly reported by Chen et al.10 This easily accessible modality enables direct visualisation of nasal cavity to assess for underlying masses while awaiting further radiological imaging. Where nasal bone involvement is likely to require reconstruction, flexible nasendoscopy is indispensible to enable surgical planning.

Numerous management options have been described in the literature, owing to great variability in clinical presentation regarding the size of the lesion, involvement of underlying bone and structural issues such as deviated nasal septum following trauma.13 Management of nasal gout does not yet have a defined treatment protocol due to its rarity and variable presentation.

The main options available are an open biopsy or transnasal approach. In our case, an intercartilaginous approach was used as this allowed good access to the soft tissue of the dorsum without causing an external scar. This involves an incision between the upper and lower alar cartilages. It has the advantage of involving and preserving the interdomal ligaments and involves the least undermining. If the tophi were present within the nasal cavity, then a direct biopsy could have been performed for diagnosis. In contrast, an open approach that involves an infracartilaginous incision and a connecting transcolumellar incision involves extensive undermining and an external scar but allows for excellent exposure, which may be necessary for more extensive disease.14

Diagnostic confirmation of nasal gout in these cases derives from postoperative tissue analysis. Kwak et al advocate the importance of fresh samples, as formalin fixation can dissolve the monosodium urate crystals.13 Management of all reported cases have produced satisfactory cosmetic results and improvement in nasal obstruction. Although the maximum duration of reported follow-up is 2 years, no recurrences have thus far been reported.8 It remains to be seen what the long-term recurrence rates are for tophaceous nasal gout.

This case report, together with the available literature, supports the early management and control of serum urate levels in reducing the incidence of acute attacks of gout and the subsequent development of gouty tophi. Once developed, prompt diagnosis and management are needed to maintain and improve quality of life.15

Learning points

  • Consider nasal gout in patients with nasal lesions and a history of gout arthropathy.

  • Strong correlation to gouty arthritis symptoms is key.

  • CT scan is a key investigation to assess for localised bony destruction.

  • Good medical control of urate levels can help reduce the development of gouty tophi.

Footnotes

  • Contributors ER: planning, confirmation of patient consent, acquisition of data, literature review and drafting of manuscript; EW: planning, editing and approval of manuscript and LM: consultant in charge of patient care, concept, planning, editing and approval of manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

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