A rare case of (Strawberry Gingivitis) associated with Wegener granulomatosis (Granulomatosis with polyangiitis GPA)

Review of the literature and case presentation

Abstract

Rapidly developing gingival hyperplasia is always an alarming periodontal condition because of being the first clinical sign of several malignant hematological diseases: notably acute myeloid, monocyter and lymphoid leukemia. This paper describes a 35-year-old female patient’s case who presented the typical form of the so called “strawberry gingivitis” that developed within two days in mid October 2016. After several detailed medical and immunological examinations the possibility of any malignant hematological diseases was excluded and therefore conventional gingivectomy was performed under local anaesthesia. The total excised tissue was sent to histology. The histology resulted in the diagnosis of a nonspecific pyogenic granuloma. After gingivectomia the gingival healing was very uneventful leading to total re-epithelialization of the gum in three weeks, and no recurrence occurred afterwards. Nevertheless, patient’s general physical condition further deteriorated and more and more organs were involved in the process. After several additional blood tests, immunological examinations and a positive test for EBV-infection a tentative diagnosis of infective mononucleosis was set up that lead to several weeks of delay in the indication of appropriate therapy. Finally, in mid January it was diagnosed as GPA (Wegener’s granulomatosis) on the basis of the plethora of characteristic clinical symptoms (strawberry gingivitis, sinus and ear infection, saddle nose, pneumonia and salivary gland swelling) but finally verified by the presence of the anti-neutrophil cytoplasmic antibody (c-ANCA test) and a positive biopsy from the submandibular salivary gland. Then the patient in very serious physical condition was admitted to the Clinic of Internal Medicine, Semmelweis University and subsequently placed on a massive corticosteroid and immunosuppressive regiments of therapy. The GPA showed an unusual generalization pattern affecting practically all tissues and also the major blood vessel in the lover extremities, leading to the halt of circulation. Due to an effective immuno-suppressive therapy combined with vasodilatation patient’s general physical condition improved, the circulation was restored in her legs, the pulmonary involvement improved and the salivary gland hyperplasia receded.
The early diagnosis is crucial in this potentially fatal disease to apply an adequate therapy and control the rapid progression and the generalization of disease. In our case there have been several weeks of delay because of the non specific histological results from the gingival biopsy and the very high circulating anti-EBV and CMV antibody titer, that predicted the possibility of infectious mononucleosis. Because of GPA’s rapid progression and potentially fatal outcome, an early diagnosis of GPA is vital and very important. Therefore, dentists should be aware of the oral signs, notably the very rare but very characteristic strawberry gingivitis and the complex symptoms of such systemic immune vasculitis. This case report shows that the gingival hyperplasia may represent an initial manifestation of an underlying systemic disease and if it properly recognized can accelerate the final diagnosis and the administration of proper therapy.