Otolaryngology Case Reports 7 (2018) 22–24

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Otolaryngology Case Reports

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Granulomatosis with polyangiitis presenting with facial nerve paralysis and T

strawberry gingivitis in a child
Michael Coulter, MDa, Daniel Hershey, MDb, Wen Jiang, MDc,∗
a
Department of Otolaryngology, Naval Medical Center, San Diego, CA, USA
b
Department of Pediatrics, University of California San Diego, Rady Children's Hospital in San Diego, San Diego, CA, USA
c
Department of Surgery – Division of Otolaryngology, University of California San Diego, Rady Children's Hospital in San Diego, San Diego, CA, USA

A R T I C LE I N FO A B S T R A C T

Keywords: Granulomatosis with Polyangiitis (GPA), formally termed Wegener's Granulomatosis, is characterized by ne-
Granulomatosis with polyangiitis crotizing granulomatous inflammation affecting the upper and lower respiratory tract and the kidneys. We
Wegener's granulomatosis report a case of a 10-year-old female who presented with a complete right facial paralysis preceded by a 6-week
Strawberry gingivitis history of intermittent fever, headaches, and a right-sided otorrhea. She was admitted for urgent placement of
Facial nerve paralysis
bilateral tympanostomy tubes and intravenous antibiotics. Despite maximal medical management, she had re-
current fever and was noted to have intermittent epistaxis and gum bleeding with erythematous gingival lesions
resembling strawberries in appearance. Gingival biopsy combined with other clinical features confirmed the
diagnosis of GPA. Strawberry gingivitis, a reddish-purple exophytic gingival swelling, is a rare presenting feature
of GPA; however, when present, it is almost pathognomonic. Early recognition of this clinical feature is critical in
establishing a timely diagnosis.

Introduction
Granulomatosis with polyangiitis (GPA), formerly known as
Wegener's granulomatosis, is a rare multisystem autoimmune disease
with features of necrotizing granulomatous inflammation involving the
upper and lower respiratory tract and inflammatory cell infiltration
leading to vasculitis in small- and medium-sized blood vessels. Onset in
early childhood is exceedingly rare, and patients often present with
nonspecific symptoms suggesting systemic inflammatory processes. We
present a case where the initial symptoms of otorrhea and facial pa-
ralysis suggested a more common infectious etiology. After failing the
standard antimicrobial therapy and clinical evidence of a systemic in-
flammatory process with multi-organ involvement, her gingival ap-
pearance prompted further work-up that lead to the eventual diagnosis
of GPA.
Case presentation
A 10-year-old Hispanic female presented to the emergency depart-
ment with a complete right facial paralysis preceded by a 6-week his-
tory of intermittent fever, headaches, and a right-sided otorrhea that
was unresponsive to oral and topical antibiotics. Family denied any
previous history of ear infections. The physical examination revealed a
complete right upper and lower division facial nerve paralysis. Otologic
exam revealed bilateral thickened tympanic membranes with purulent
right-sided otorrhea. Computed tomography (CT) of the temporal bone
showed bilateral opacified middle ear spaces and mastoid air cells, right
worse than left without coalescence. She was admitted for urgent pla-
cement of bilateral tympanostomy tubes, intravenous antibiotics and
steroid administration.
She was discharged home after the resolution of her fever, but was
readmitted in 7 days with recurrent fever up to 103 °F. Her white count
was 14,000/μl [reference range 4500-10,000/μl]. Serum inflammatory
markers were markedly elevated with ESR of 63 mm [reference range
0–20 mm] and CRP of 21.3 mg/dL [reference range 0–0.99 mg/dL].
Patient also had microhematuria and proteinuria on urine analysis. At
this time, the family reported gum bleeding and intermittent epistaxis,
both of which had been present since the onset of the initial fever. She
was noted to have erythematous exophytic gingival lesions with pete-
chial hemorrhages resembling strawberries (Fig. 1). Gingival biopsy
revealed non-keratinizing stratified squamous epithelium with a poly-
poid and pseudoepitheliomatous hyperplasia, with focal ulceration and
a dense accumulation of neutrophils with a smaller number of lym-
phocytes and plasma cells (Fig. 2). Patient also underwent kidney
biopsy that demonstrated C3 immunofluorescence and electron micro-
scopic evidence of vasulopathy without frank necrotizing

∗
Corresponding author. Department of Surgery, Division of Otolaryngology, Rady Children's Hospital, 3020 Children's Way MC 5024, San Diego, CA 92123, USA.
E-mail addresses: Michael.jack.coulter@gmail.com (M. Coulter), dhershey@rchsd.org (D. Hershey), wjiang@rchsd.org (W. Jiang).

https://doi.org/10.1016/j.xocr.2018.05.002
Received 17 March 2018; Accepted 3 May 2018
Available online 06 May 2018
2468-5488/ Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/).
M. Coulter et al.
Fig. 1. Characteristic appearance of Strawberry Gingivitis with lobulated,
reddish swelling involving the gingival mucosa and petechial hemorrhages
resembling strawberries.
Fig. 2. H&E histology of gingival biopsy at 200× magnification demonstrating
non-keratinizing stratified squamous epithelium with a polypoid and pseu-
doepitheliomatous hyperplasia, with focal ulceration and a dense accumulation
of neutrophils with a smaller number of lymphocytes and plasma cells.
glomerulonephritis. Serum anti-proteinase-3 (PR3) antibodies were
markedly elevated. CT of the chest showed multiple lobular low at-
tenuation lesions emanating from the left hilum and extending into the
left infrahilar region along a bronchovascular distribution suggestive of
caseating or necrotizing lympadenopathy (Fig. 3).
Patient was started on methylprednisolone 30mg twice daily with
rapid resolution of fever, improvement of her gingival bleeding, and
normalization of inflammatory markers. She was gradually tapered off
of steroid on an outpatient basis and started on cyclophosphamide as a
maintenance therapy. She now has a House-Brackman grade II (very
mild) weakness of the right marginal branch, while the rest of the facial
nerve function has fully recovered.
Discussion
Patient's initial presentation suggested a more common infectious
etiology. With her recurrent fever and the classic appearance of
strawberry gingivitis, further work-up of a systemic inflammatory
process was pursued. Although the gingival histologic finding was not
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Otolaryngology Case Reports 7 (2018) 22–24
Fig. 3. Multiple lobular low attenuation lesions emanating from the left hilum
and extending into the left infrahilar region along a bronchovascular distribu-
tion with the largest measuring 2.2 × 2.5 × 2.4cm. These lesions are suggestive
of caseating or necrotizing lymphadenopathy that is consistent with the pre-
sentation of GPA.
completely specific, combined with her clinical history, laboratory re-
sults, imaging and renal biopsy, we arrived at the diagnosis of
Granulomatosis with Polyangiitis (GPA), formerly termed “Wegener's
Granulomatosis”. GPA is characterized by the classic triad of necro-
tizing granulomas affecting the upper (including ears and nose) and
lower respiratory tracts, disseminated vasculitis, and glomerulone-
phritis. Diagnosis is based on clinical manifestations as well as histo-
logical evidence of necrotizing vasculitis or granulomatous inflamma-
tion.
GPA may present at any age; among children, it is more common in
females in early teens. It is hypothesized to be associated with en-
vironmental and infectious triggers in genetically predisposed in-
dividuals. Strawberry gingivitis, a reddish-purple exophytic gingival
swelling, is a rare presenting feature of GPA that occurs in only 2% of
cases. However, when present this sign is almost pathognomonic for
GPA [1] and recognition is important for a timely diagnosis and
treatment as oral lesions often persist for a long time before multi-organ
involvement occurs [2,3]. Cranial nerve neuropathy is seen in less than
5% of GPA cases, but an inflammatory condition should be considered
in this case with the constellation of systemic involvement that cannot
be explained by complicated acute otitis media.
Although antineutrophil cytoplasm antibodies (ANCA) positivity is
not essential for diagnosis and may be negative in up to 20% of cases,
these antibodies play an important role in pathogenesis [4]. ANCA is a
useful diagnostic marker in a range of small vessel vasculitides in-
cluding GPA, microscopic polyangiitis, Churg-Strauss syndrome, and
renal-limited vasculitis. They may be seen by indirect immuno-
fluorescence of neutrophils in a distinct cytoplasmic or perinuclear
fluorescence (c-ANCA and p-ANCA respectively). C-ANCA antibodies
are generally directed against proteinase 3 (PR3) and p-ANCA against
myeloperoxidase antigens in granules of neutrophils, detected by en-
zyme-linked immunosorbent assay (ELISA). It is recommended that
both IIF and ELISA are used for detection of ANCA [5]. C-ANCA is
positive in up to 90% of patients with active GPA, and PR3 antibodies
are found in 90% of GPA patients with a positive ANCA [4]. Positivity
in both c-ANCA and PR3 yields a specificity of 99% [6].
Recommended medical management is based on severity.
Corticosteroids are first line options for acute treatment; methotrexate
is commonly added for milder disease while cyclophosphamide or ri-
tuximab is reserved for severe disease [7].
M. Coulter et al.
Conclusion
GPA is an uncommon diagnosis in the pediatric population and may
present with a varied combination of non-specific clinical features de-
pending on what organs are involved. This patient presented with facial
nerve paralysis and strawberry gingivitis, both of which are rare pre-
senting features of GPA. In this case, combined with other systemic
manifestation, they lead to the eventual diagnosis of GPA. Early re-
cognition of these clinical features is critical in establishing a timely
diagnosis and initiate appropriate treatment.
Funding
No financial support was received for this work.
Conflicts of interest
None.
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Otolaryngology Case Reports 7 (2018) 22–24
Appendix A. Supplementary data
Supplementary data related to this article can be found at http://dx.
doi.org/10.1016/j.xocr.2018.05.002.
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