Cheilitis granulomatosa

CASE REPORT

A 47-year-old female patient presented with a chief complaint of swelling in the upper lip since 2 months which had gradually increased in size. History revealed that the patient had visited a dentist 1 month ago with a complaint of broken upper left lateral incisor that was extracted, since it was considered to be the possible source of trauma. There was no subsequent change in swelling after extraction. There was no history of habits and medical history revealed that the patient was on antihistamines and prednisolone after which the lesion regressed for about a week and recurred again. There were no associated signs and symptoms.

On examination, a solitary, ovoid swelling was noted on upper labial mucosa, measuring approximately 3 × 2 cm, with diffuse borders. The skin and labial mucosa appeared dry and scaly. On palpation, the swelling was firm, non-tender and nodular. A single submandibular lymph node was palpable, mobile and tender.

On gross examination, the specimen was roughly wedge-shaped, creamish white in color, measured 1.1 × 0.7 cm in size, with a smooth surface and was firm to rubbery in consistency.

A provisional diagnosis of Orofacial Granulomatosis was made.

Microscopically, the section showed stratified squamous epithelium exhibiting pseudoepitheliomatous hyperplasia with areas of para and orthokeratinization [Figures 1 and 2]. The underlying connective tissue showed organized non-caseating granulomas containing epithelioid cells, multinucleated giant cells of Langhans type with nine to thirteen nuclei arranged peripherally, surrounded by lymphocytes and plasma cells [Figures 3–5]. These granulomas were surrounded by collagen fibers interspersed with fibroblasts and fibrocytes. The stroma was edematous and contained intense, perivascular chronic inflammatory cell infiltrate. Multiple peripheral nerve twigs were found and were suggestive of peripheral neuropathy [Figure 6].

Figure 1.

Stereomicroscopic image showing superficial stratified squamous epithelium and underlying connective tissue stroma forming numerous organized granulomas lacking central areas of caseation. (H&E stain, ×20)

Figure 2.

Stratified squamous epithelium exhibiting pseudoepitheliomatous hyperplasia with rich fibrocellular and fibrovascular connective tissue stroma. (H&E stain, ×40)

Figure 3.

Multiple non-caseating granulomas surrounded by collagen fibers and small blood vessels. (H&E stain, ×40)

Figure 5.

Organized non-caseating granulomas containing epithelioid cells, multinucleated giant cells of langhans type surrounded by inflammatory cells (H&E stain, ×200)

Figure 6.

Interspersed nerve twigs surrounded by chronic inflammatory cells (H&E stain, ×200)

Figure 4.

Multinucleated giant cells of Langhans type with nine to thirteen nuclei arranged peripherally, surrounded by epithelioid cells, lymphocytes, plasma cells and blood vessels. (H&E stain, ×100)

Based on these features and by correlating with the clinical findings, a final diagnosis of cheilitis granulomatosa was made.

Differential diagnosis

Crohn's disease

Crohn's disease is a slowly progressive disease characterized by granulomatous superficial ulcerations of the gastrointestinal tract.[1] Symptoms include abdominal cramps, colicky pain, alternating constipation and diarrhea.[2] Microscopically isolated, loose, poorly formed non-caseating granulomas are found deeply situated with occasional giant cells, scanty inflammatory cells and fibrosis.[3] We excluded this lesion as no gastrointestinal symptoms were present.

Sarcoidosis

Is a multisystem granulomatous disease, presents with hilar lymphadenopathy, pulmonary infiltration and skin and eye lesions. Microscopically, proliferative, non-caseating granulomas with epithelioid cells and multinucleated giant cells are seen.[1] Often laminated basophilic calcifications known as Schaumann bodies, stellate inclusions known as asteroid bodies and Hamazaki-Wesenberg bodies are found. We excluded sarcoidosis as it lacked histological and clinical evidence.[4]

Foreign body giant cell granuloma

Structures that cannot be digested by tissue macrophages evoke a cascade of inflammatory reactions.[5] They form foreign body granulomas with giant cells and which are often seen on routine hematoxylin and eosin (H and E) sections.[6] This was excluded based on the history, clinical and histopathological features.

Atypical tuberculosis

Microscopically, granulomas show circumscribed collections of epithelioid histiocytes, lymphocytes, multinucleated giant cells and central caseation necrosis. This was excluded as there were no other clinical symptoms or involvement of other sites.