Enlarged red gums in a 42-year-old patient with no significant past medical history
A 42-year-old woman came to the dermatology clinic with complaints of painful enlargement of her gums for the past 6 weeks and ulcer on her face for the past 4 weeks. The patient revealed that her gingival hyperplasia was rapidly progressive and painful too. Upon inquiring, the patient also told that she had recurrent episodes of bleeding from the nose (epistaxis).
On examination, three necrotic ulcers were noticed on his face, and oral examination showed gingival hyperplasia with a granular and hemorrhagic appearance typical of “strawberry gingivitis.”
Serological investigations showed an increased level of antineutrophil cytoplasmic antibodies (ANCA) with a cytoplasmic staining pattern (c-ANCA). Enzyme-linked immunosorbent assay (ELISA) for anti–proteinase 3 antibodies was positive.
The rest of the laboratory tests were normal, including the serum creatinine levels and the urinalysis.
Normal paranasal sinuses were appreciated on the computed tomography of the head, whereas the CT scan of the chest showed multiple pulmonary nodules.
Based on clinical and laboratory findings, a diagnosis of granulomatosis with polyangiitis.
Treatment with prednisone and cyclophosphamide was started, but the patient never followed up.
Formerly known as Wegener’s granulomatosis, granulomatosis with polyangiitis is an uncommon disorder in which the small- to medium-sized vessels of the body get inflamed and the organs that these vessels supply get affected. This rare autoimmune disorder has characteristic necrotizing granulomas along with recurrent respiratory infections, renal abnormalities, and constitutional symptoms, including weight loss and fever.
Gingival hyperplasia or strawberry gingivitis is a rare manifestation of granulomatosis with polyangiitis, but when present, is highly suggestive of the disease.
The most definitive test for diagnosis is tissue biopsy (lung or renal), but it is not always necessary. Laboratory investigations, including the c-ANCA levels and clinical correlation, maybe enough for establishing a working diagnosis.
If and when a renal biopsy is performed, the most common finding is segmental crescentic necrotizing glomerulonephritis with little or no immunoglobulin or complement deposition (pauci-immune)
Corticosteroids and cytotoxic agents remain the mainstay of treatment for granulomatosis with polyangiitis; if left untreated, the prognosis is quite poor.
References
Maryam Ghiasi, M. (2017, November 23). Strawberry Gingivitis in Granulomatosis with Polyangiitis. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1706987
Heera R, Choudhary K, Beena VT, Simon R. Strawberry gingivitis: A diagnostic feature of gingival Wegener’s granulomatosis!. Dent Res J (Isfahan). 2012;9(Suppl 1): S123-S126.
