Riga-Fede disease: a mimicker of malignancy

Abstract

Riga-Fede disease (RFD), also known as traumatic eosinophilic granuloma, is a benign inflammatory condition of the oral cavity that may mimic a malignant tumour. It’s a rare condition mainly reported in infants. We present a unique case report of a 57-year-old man who presented to our outpatient department with foreign body sensation in the throat and swelling over the dorsum of the tongue for a period of 1 month. He gave no history of trauma, especially due to sharp teeth. Video laryngoscopy done revealed an ulceroproliferative growth on the midline of the dorsum of the tongue. With clinical suspicion of malignancy, the patient underwent wide local excision of the tongue lesion. Histopathological examination was suggestive of RFD. The postoperative follow-up was uneventful with good wound healing. The patient was followed up with no evidence of recurrence.

Background

After its first histopathological description in 1890, Riga-Fede disease (RFD) has been referred to by many names: ulcerative eosinophilic granuloma, traumatic granuloma, sublingual granuloma, to name a few.¹ Later, in 1983, Elzay described a similar chronic reactive ulceration of oral mucosa referring to it as ‘traumatic ulcerative granuloma with stromal eosinophilia’.¹ Even though rare, it has commonly been noted in neonates and infants.² However, there have been reports of Riga-Fede-like diseases being noted in adults, especially in the immunocompromised.³ The underlying aetiology has been suspected as trauma, especially due to sharp dentition. However, such association has been noted only in 39% of reported cases.^{3 4} When occurring in adults, it can often be mistaken as a malignant ulcer. Riga-Fede is usually associated with pain and is self-limiting, unlike malignant ulcers, which are painless and non-healing.⁵

Case presentation

A 57-year-old man, known diabetic and hypertensive, presented to our outpatient clinic with reports of progressively increasing swelling over the tongue, and foreign body sensation in the throat for 1 month. He gave no history of repeated trauma to the tongue. Oral examination revealed a 3×3 cm painless ulceroproliferative growth over the dorsal surface of the tongue, extending anteriorly 5 cm from the tip of the tongue, laterally 2 cm from the lateral border of the tongue on each side and posteriorly just anterior to the level of the anterior pillar (figure 1). The floor of the ulcer was covered with a central necrotic slough. The patient had good orodental hygiene with no sharp tooth. Indirect laryngoscopy revealed no extension to the base of the tongue and normal laryngopharynx.

Figure 1.

Video laryngoscopy picture showing an ulceroproliferative growth with central necrotic slough over the midline of the dorsum of the tongue.

Investigations

The patient underwent video laryngoscopic examination that showed a 3×3 cm ulceroproliferative growth over the midline of the dorsum of the tongue (figure 1).

Histopathology with 100× magnification showed areas of granulation tissue formation with abundant eosinophils (figure 2).

Figure 2.

H&E stain at 100× magnification showing areas of granulation tissue formation with abundant eosinophils.

Magnification to 200× showed hyperplastic squamous epithelium with areas of ulceration (figure 3).

Figure 3.

H&E stain at 200× magnification showing hyperplastic squamous epithelium with areas of ulceration.

Differential diagnosis

Presenting history with age, absence of trauma, comorbidities and clinical findings were strongly suggestive of malignancy. However, features of absent neck nodes and no induration around the lesion were the only factors against possible malignancy.

Treatment

We performed a wide excision of the lesion, and tissue sent for histopathological examination revealed hyperplastic squamous epithelium with areas of ulceration (figure 2). The subepithelium showed numerous inflammatory cells composed of eosinophils, neutrophils and plasma cells, along with areas of granulation tissue formation (figure 3). There were no features of malignancy seen and hence a histopathological diagnosis of RFD was made.

Outcome and follow-up

The patient was followed up at 1 year with good wound healing and no signs of recurrence.

Discussion

RFD is mainly a histopathological diagnosis with a suspected traumatic aetiology.^{2 3} The condition is most commonly observed in children and the occurrence of the lesion usually coincides with the eruption of primary teeth.^2–4 However, there have been a few cases reported in older children and adults, especially in those immunocompromised. Our case was an elderly man, a known diabetic and hypertensive. Initially, exclusively reported as ulceration over the lingual frenulum of infants, it is now known to involve any part of the oral cavity mucosa.⁵

However, our case presented with a non-healing ulceroproliferative growth involving the midline of the dorsal surface of the tongue with a central necrotic slough. Such features seemed characteristic of a malignant mucosal lesion and unlike the expected presentation of RFD.

The microscopic picture of traumatic ulcers of inflammatory cells with areas of ulceration is similar to that in our patient.^{5 6} The causes of trauma differ in the adult population as they may be related to the presence of broken teeth or ill-fitting prosthetic material in the oral cavity. However, our case had no such history of trauma.

Clinically, it can be commonly mistaken with malignancy and hence a tissue diagnosis is imperative for confirmation.^5–7 Our case underwent wide excision, and tissue sent for histopathological examination ruled out malignancy and established a diagnosis of Riga-Fede.

Histological features of RFD of infiltration by various inflammatory cells may seem non-specific. Characteristic histological features in RFD are the stomal infiltration by eosinophils and mast cells. A similar picture was seen in our case—ulcerated hyperplastic epithelium with increased stromal eosinophil and mast cell infiltration.

The clinical picture of RFD is non-specific. Hence the clinical features may overlap with various conditions from benign infections, mucositis or glossitis to more aggressive malignant mucosal lesions (secondary to leukaemia, lymphoma or squamous cell carcinoma).^6–9 For this reason, a clinician must be familiar with such rare entities as RFD.

A review of the literature shows the varied treatment modalities used for RFD. Extraction of sharp teeth may be avoided and smoothening of only the sharp edges may suffice.⁵ However, the limitations of tooth restoration in such a scenario may be accidental ingestion or inhalation of composite resin used for restoration. Hence the risk–benefit of avoiding tooth extraction, especially in children, needs assessment. The majority of authors have described the treatment as simple wide excision, as done for our case.^{1 5} All reported cases have been known to heal without recurrence, which is also similar to our case.

Patient’s perspective.

I first visited the hospital with a foreign body sensation in the throat for about a month or two when about a month ago I noticed a small swelling on my tongue, which gradually kept increasing in size. The fact that I did not have any pain or any difficulty swallowing food, kept me at bay for a month, but the increase in size started worrying me. So decided to go and get myself checked.

The doctor was so patient, compassionate listened to my words and then examined my tongue completely, and then suggested me to undergo a video laryngoscopy, which I was a little apprehensive about. The doctor told me I had quite a big swelling on the tongue, but the other parts of my throat until the voice box was normal. However, because of my age and increasing size, and my comorbid conditions, doctors suggested me to go ahead with the surgery and the pathological diagnosis is a must. The surgery was uneventful I recovered well. I was getting scared for 2 days until my reports came. To all our surprise, including the doctors, I was diagnosed with a rare disease called Riga-Fede. However, doctors explained to me it is a benign condition and no further treatment was needed, and advised for regular follow-up. At present, I am very comfortable with no other problems.

Learning points.

• Due to non-specific clinical presentation, Riga-Fede disease (RFD) may overlap with mucosal ulcerative lesion with varied aetiology—infective (cytomegalovirus, syphilitic chancre, tuberculosis or fungal disease) to inflammatory (amyloidosis) to more sinister malignancy like leukaemia, lymphoma and squamous cell carcinoma.

• Clinician should be familiar with a rare clinical entity, such as RFD.

• Histopathological analysis is essential for confirmation of diagnosis.

• Although the lesion may appear aggressive and sinister, a benign and simple excision with follow-up is adequate.